Description

Strain Information

Type Inbred Strain; Additional information on Inbred Strains. Visit our online Nomenclature tutorial. Mating System Sibling x Sibling         (Female x Male)   01-MAR-06 Breeding Considerations This strain is a good breeder. Species laboratory mouse H2 Haplotype d Generation F241pF249 (17-SEP-12) Generation Definitions

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Appearance
albino
Related Genotype: A/A Tyrp1^b/Tyrp1^b Tyr^c/Tyr^c

Important Note
This strain is homozygous for Cdh23^ahl, the age related hearing loss 1 mutation, which on this background results in progressive hearing loss with onset after 10 months of age.

Description
BALB/c mice are particularly well known for the production of plasmacytomas following injection with mineral oil, forming the basis for the production of monoclonal antibodies. Although not all BALB/c substrains have been examined for plasmacytoma induction, substrains derived from the Andervont (An) lineage (which includes BALB/cByJ) typically are susceptible, while those descended from BALB/cJ are resistant (see: Potter M ,1985). Mammary tumor incidence is normally low, but infection with mammary tumor virus by fostering to MMTV⁺ C3H mice dramatically increases tumor number and age of onset. BALB/c mice develop other cancers later in life including reticular neoplasms, primary lung tumors, and renal tumors. Rare spontaneous myoepitheliomas arising from myoepithelial cells of various exocrine glands have been observed in both BALB/cJ and BALB/cByJ substrains. BALB/cByJ has a deletion in the Qa2 subregion of the murine MHC.

Related Strains

BALB Strains

000650	BALB/cBy
001905	BALB/cGaJ
000921	BALB/cGrRkJ
000651	BALB/cJ
001311	BALB/cWtEiJ

View BALB Strains     (5 strains)

Strains carrying   Ahr^b-2 allele

000645	A/HeJ
000646	A/J
000130	B6.C-H17c/(HW14)ByJ
000370	B6.C-H38c/(HW119)ByJ
000653	BUB/BnJ
000659	C3H/HeJ
000656	CBA/J
000657	CE/J
000352	CXB2/ByJ
000353	CXB3/ByJ
000354	CXB4/ByJ
000355	CXB5/ByJ
000357	CXB7/ByJ
000673	HRS/J
000679	P/J
000930	PERA/EiJ
000644	SEA/GnJ
000280	SF/CamEiJ

View Strains carrying   Ahr^b-2     (18 strains)

Strains carrying   Cdh23^ahl allele

001137	129P1/ReJ
000690	129P3/J
000691	129X1/SvJ
000646	A/J
000647	A/WySnJ
003070	ALR/LtJ
003072	ALS/LtJ
004502	B6;AKR-Lxl2/GrsrJ
000653	BUB/BnJ
005494	C3.129S1(B6)-Grm1rcw/J
000664	C57BL/6J
004764	C57BL/6J-Cdh23v-8J/J
003129	C57BL/6J-Epha4rb-2J/GrsrJ
004820	C57BL/6J-Kcne12J/J
004703	C57BL/6J-Kcnq2Nmf134/J
004811	C57BL/6J-nmf110/J
004812	C57BL/6J-nmf111/J
004747	C57BL/6J-nmf118/J
004656	C57BL/6J-nmf88/J
004391	C57BL/6J-Chr 13A/J/NaJ
004385	C57BL/6J-Chr 7A/J/NaJ
000662	C57BLKS/J
000667	C57BR/cdJ
000668	C57L/J
000669	C58/J
010614	CBACa.B6-Cdh23ahl/Kjn
000657	CE/J
000670	DBA/1J
001140	DBA/1LacJ
000671	DBA/2J
007048	DBA/2J-Gpnmb+/SjJ
002106	KK/HlJ
000675	LG/J
000676	LP/J
000677	MA/MyJ
001976	NOD/ShiLtJ
002050	NOR/LtJ
000679	P/J
002747	SENCARB/PtJ
002335	SKH2/J
003392	STOCK Crb1rd8/J

View Strains carrying   Cdh23^ahl     (41 strains)

Strains carrying   Hld allele

000651	BALB/cJ
000351	CXB1/ByJ
000353	CXB3/ByJ
000354	CXB4/ByJ
000355	CXB5/ByJ
000357	CXB7/ByJ

View Strains carrying   Hld     (6 strains)

Strains carrying other alleles of Ahr

000690	129P3/J
000648	AKR/J
002920	B6(D2N).Spretus-Ahrb-3/J
006203	B6.129(FVB)-Ahrtm3.1Bra/J
002831	B6.129-Ahrtm1Bra/J
000136	B6.C-H34c/(HW22)ByJ
008599	B6.Cg-Cyp1a2/Cyp1a1tm2Dwn Ahrd Tg(CYP1A1,CYP1A2)1Dwn/DwnJ
002921	B6.D2N-Ahrd/J
002727	B6;129-Ahrtm1Bra/J
000652	BDP/J
000663	C57BL/6By
001139	C57BL/6ByJ
000664	C57BL/6J
000662	C57BLKS/J
000667	C57BR/cdJ
000668	C57L/J
000669	C58/J
000926	CAROLI/EiJ
000928	CAST/EiJ
000351	CXB1/ByJ
000356	CXB6/ByJ
002937	D2.B6-Ahrb-1/J
000671	DBA/2J
000674	I/LnJ
000675	LG/J
000676	LP/J
000677	MA/MyJ
000550	MOLF/EiJ
000684	NZB/BlNJ
000726	RBF/DnJ
000682	RF/J
000686	SJL/J
001146	SPRET/EiJ
000688	ST/bJ
000689	SWR/J
000693	WC/ReJ KitlSl/J
000933	YBR/EiJ

View Strains carrying other alleles of Ahr     (37 strains)

Strains carrying other alleles of Cdh23

002552	B6(V)-Cdh23v-2J/J
002756	B6.CAST-Cdh23Ahl+/Kjn
010615	B6.CBACa-Cdh23CBA/CaJ/Kjn
002432	B6J x B6.C-H2-Kbm1/ByJ-Cdh23v-J/J
004764	C57BL/6J-Cdh23v-8J/J
004819	C57BL/6J-Cdh23v-9J/J
005016	CByJ;B6-Cdh23v-10J/J
000275	V/LeJ

View Strains carrying other alleles of Cdh23     (8 strains)

Strains carrying other alleles of Mdmg1

000360

B6.C-H2d Mdmg1BALB/cBy/aByJ

View Strains carrying other alleles of Mdmg1     (1 strain)

Additional Web Information

JAX^® NOTES, April 1988; 433. H-2 Haplotypes of Mice from Jackson Laboratory Production Colonies.
JAX^® NOTES, Fall 1990; 443. A Brief History of the Two Substrains of BALB/c, BALB/cJ, and BALB/cByJ Available from Animal Resources.
JAX^® NOTES, Fall 1990; 443. Ulcerative Blepharitis and Periorbital Abscesses in BALB/cJ and BALB/cByJ Mice.
JAX^® NOTES, July 1987; 430. BALB/cJ vs. BALB/cByJ.
JAX^® NOTES, Spring 2003; 489. Malocclusion in the Laboratory Mouse.
JAX^® NOTES, Summer 1992; 450. Myoepitheliomas in Inbred Laboratory Mice.
JAX^® NOTES, Summer 2003; 490. Hydrocephalus in Laboratory Mice.
JAX^® NOTES, Winter 2006; 504. JAX^® Mice: the Gold Standard Just Got Better.
Mouse Phenome Database / SNP Facility

Phenotype

Phenotype Information

View Phenotypic Data

Phenotypic Data

Body Weight Information - JAX^® Mice Strain BALB/cByJ (001026)
(This chart reflects the typical correlation between body weight and age for mice maintained in production colonies at The Jackson Laboratory.)

Mouse Phenome Database
Festing Inbred Strain Characteristics: BALB/c
JAX^® Physiological Data Summary [pdf]
JAX^® Physiological Data Protocol [pdf]

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI

- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).

Acyl-Coa Dehydrogenase, Short-Chain, Deficiency Of; ACADSD

- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested. Deafness, Autosomal Recessive 12; DFNB12   (CDH23) Usher Syndrome, Type ID; USH1D   (CDH23)

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI

      assigned by genotype

Acads^del-J/Acads^del-J

        BALB/cByJ

• behavior/neurological phenotype
• abnormal food preference
  • given a choice, selection of a fat/protein diet declines with time   (MGI Ref ID J:87779)
• abnormal liquid preference
  • did not develop a preference for drinking water containing a corn oil emulsion   (MGI Ref ID J:87779)
• homeostasis/metabolism phenotype
• abnormal adaptive thermogenesis
  • body temperature in most BALB/cByJ mice dropped 10 degrees C in less than 4 h at 4 degrees C, with none able to maintain body temperature for longer than 8 h in the cold   (MGI Ref ID J:50729)
• abnormal circulating amino acid level
  • serum glycine concentrations are markedly lower than controls   (MGI Ref ID J:14707)
• • decreased circulating carnitine level
    • slightly lower plasma carnitine level   (MGI Ref ID J:14707)
• abnormal fatty acid oxidation
  • block in short-chain fatty acid oxidation shown by reduced butyryl-CoA dehydrogenation in liver mitochondria   (MGI Ref ID J:14707)
• aciduria
  • organic aciduria at 7-14 weeks of age, caused by relatively large amounts of n-butyrylglycine and ethylmalonate in the urine   (MGI Ref ID J:9743)
  • ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher than in controls   (MGI Ref ID J:4165)
  • markedly increased urinary concentrations of ethylmalonic and methylsuccinic acids, and N-butyrylglycine in nonfasting and fasting mice, and remained elevated with or without medium chain triglyceride challenge   (MGI Ref ID J:14707)
  • upon dosing with carnitine, mice excrete large amounts of butyryl-carnitine in the urine   (MGI Ref ID J:14707)
• hypoglycemia
  • hypoglycemia develops after 18 hours of fasting with mean serum glucose measuring less than half of control values   (MGI Ref ID J:14707)
• increased circulating HDL cholesterol level
  • HDL levels were significantly increased in mutant mice for both males and females fed either a regular chow or a high-fat diet   (MGI Ref ID J:164460)
• liver/biliary system phenotype
• hepatic steatosis
  • fat deposits accumulate in the liver after 18 hours of fasting or with dietary fat challenge   (MGI Ref ID J:14707)
• renal/urinary system phenotype
• aciduria
  • organic aciduria at 7-14 weeks of age, caused by relatively large amounts of n-butyrylglycine and ethylmalonate in the urine   (MGI Ref ID J:9743)
  • ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher than in controls   (MGI Ref ID J:4165)
  • markedly increased urinary concentrations of ethylmalonic and methylsuccinic acids, and N-butyrylglycine in nonfasting and fasting mice, and remained elevated with or without medium chain triglyceride challenge   (MGI Ref ID J:14707)
  • upon dosing with carnitine, mice excrete large amounts of butyryl-carnitine in the urine   (MGI Ref ID J:14707)
• cellular phenotype
• abnormal fatty acid oxidation
  • block in short-chain fatty acid oxidation shown by reduced butyryl-CoA dehydrogenation in liver mitochondria   (MGI Ref ID J:14707)
• muscle phenotype
• abnormal skeletal muscle morphology
  • wehn dosed with carnitine, mutant muscle exhibits a 9-fold increase in butyryl-carnitine concentration compared to controls   (MGI Ref ID J:14707)

Hld/Hld

        either: BALB/cByJ or BALB/cJ

• nervous system phenotype
• abnormal dendrite morphology
  • abnormally positioned by being late-generated   (MGI Ref ID J:12029)
  • cell bodies are located below the intrapyramidal mossy fiber layer   (MGI Ref ID J:12029)
• abnormal hippocampus morphology   (MGI Ref ID J:13989)
• • abnormal hippocampal mossy fiber morphology   (MGI Ref ID J:12029)
  • abnormal hippocampus CA3 region morphology
    • there is abnormal lamination of this cell layer resulting in superficial early generated neurons and deep late-generated neurons   (MGI Ref ID J:12029)
  • abnormal hippocampus layer morphology
    • the anomaly is also seen in BALB/cByJ, F1 mice involving BALB sublines noted, and some CXB RI lines; C57BL/6J is considered wild-type for this feature   (MGI Ref ID J:13989)
    • this is secondary to a defect in neuronal migration   (MGI Ref ID J:13989)
  • • ectopic hippocampus pyramidal cells
      • located below the intrapyramidal layer; some of the ectopic cells have short, fine-caliber dendritic branches arising near the points of contact of the intrapyramidal mossy fibers   (MGI Ref ID J:12029)
• abnormal neuronal migration   (MGI Ref ID J:13989)
• cellular phenotype
• abnormal neuronal migration   (MGI Ref ID J:13989)

View Research Applications

Research Applications

This mouse can be used to support research in many areas including:

Cancer Research
Increased Tumor Incidence
      Mammary Gland Tumors
      Mammary Gland Tumors: late onset

Immunology, Inflammation and Autoimmunity Research
Autoimmunity
      experimental allergic encephalomyelitis (EAE)

Neurobiology Research
Hearing Defects
      Age related hearing loss
Neurodevelopmental Defects
      callosal agenesis, incomplete penetrance

Research Tools
Cancer Research
      monoclonal antibodies, myeloma and hybridoma production
General Purpose
Immunology and Inflammation Research
      background strain for histocompatibility congenics

Sensorineural Research
Hearing Defects
      Age related hearing loss

Cdh23^ahl related

Neurobiology Research
Hearing Defects
      Age related hearing loss

Sensorineural Research
Hearing Defects
      Age related hearing loss

Genes & Alleles

Gene & Allele Information provided by MGI

Allele Symbol		Acadsdel-J
Allele Name		deletion, Jackson
Allele Type		Spontaneous
Common Name(s)		Bcd-1c; Scad;
Strain of Origin		BALB/cByJ
Gene Symbol and Name		Acads, acyl-Coenzyme A dehydrogenase, short chain
Chromosome		5
Gene Common Name(s)		ACAD3; AI196007; Bcd-1; Bcd1; HDL QTL 8; Hdlq8; SCAD; butyryl CoA dehydrogenase 1; expressed sequence AI196007;
Molecular Note		A 278bp deletion occurs which starts in intron "A" and includes exon "B" and the first 78bp of exon "C". As a result of this deletion, mRNA transcription is reduced to about 35% of normal and includes two forms, a longer normal length transcript and a shorter misspliced form. If these mRNAs were translated they both would result in truncated products. Protein product is undetectable immunologically and there is a total absence of enzyme activity. [MGI Ref ID J:12776] [MGI Ref ID J:50729]
Allele Symbol		Ahrb-2
Allele Name		b-2 variant
Allele Type		Not Applicable
Common Name(s)		Ahb-2; Ahh;
Strain of Origin		BALB/cBy
Gene Symbol and Name		Ahr, aryl-hydrocarbon receptor
Chromosome		12
Gene Common Name(s)		Ah; Ahh; Ahre; In; aromatic hydrocarbon responsiveness; aryl hydrocarbon hydroxylase; bHLHe76; dioxin receptor; inflammatory reactivity;
General Note		C57BL/6 carries the responsive Ahrb allele; DBA/2 carries nonresponsive Ahrd. Heterozygotes (Ahrb/Ahrd) are responsive (J:5282). Later work identified a second (J:8895) and later a third (J:22144) allele conferring response. Thus the allele in C57, C58, and MA/My strains is now Ahrb-1; Ahrb-2 is carried by BALB/cBy, A, and C3H; and Ahrb-3 by Mus spretus, M. caroli, and MOLF/Ei. The nonresponsive strains AKR, DBA/2, and 129 carry Ahrd (J:22144). Nucleotide and amino acid sequence differences between Ahrb-1 and Ahrd have been determined (J:17460). Strain of origin - this allele was found in BALB/cByJ, A/J, C3H/HeJ, CBA strains
Molecular Note		This allele encodes a high affinity, heat labile, 104 kDa receptor containing 848 amino acids. Sequencing studies of cDNA from C57BL/6J congenic mice homozygous for this allele identified nucleotide substitutions in the ORF that would cause 5 amino acid differences between the C57BL/6J and BALB/cBy peptides, and 2 amino acid differences between the BALB/cBy and DBA/2J peptides. A T to C transition in exon 11 replaces the opal termination codon in the C57BL/6J allele with an arginine codon in the BALB/cByallele. This change would extend translation of the BALB/cBy mRNA by 43 amino acids, accounting for the larger size of the peptide produced by this allele (104 kDa, vs 95 kDa for the C57BL/6J allele). [MGI Ref ID J:15153] [MGI Ref ID J:22144]
Allele Symbol		Cdh23ahl
Allele Name		age related hearing loss 1
Allele Type		QTL
Common Name(s)		Cdh23753A; mdfw;
Strain of Origin		multiple strains
Gene Symbol and Name		Cdh23, cadherin 23 (otocadherin)
Chromosome		10
Gene Common Name(s)		4930542A03Rik; CDHR23; RIKEN cDNA 4930542A03 gene; USH1D; W; age related hearing loss 1; ahl; bob; bobby; bus; bustling; mdfw; modifier of deaf waddler; neuroscience mutagenesis facility, 112; neuroscience mutagenesis facility, 181; neuroscience mutagenesis facility, 252; nmf112; nmf181; nmf252; sals; salsa; v; waltzer;
Molecular Note		Genetic complementation tests have shown allelism between the mdfw (modifier of deaf waddler) locus and the ahl locus. Further analysis has identified an association between ahl and a G to A transition at nucleotide position 753 of Cdh23. This hypomorphic allele causes in frame skipping of exon 7 and reduced message stability. Twenty-seven strains classified with ahl and carrying the 753A allele include: CD1, RBF/DnJ, PL/J, AKR/J, RF/J, BALB/cBy, A/WySnJ, P/J, SENCARA/PtJ, DBA/1J, ALS/LtJ, C58/J, C57BLKS/J, 129P1/ReJ, C57BR/cd, SKH2/J, BUB/Bn, MA/MyJ, LP/J, 129X1/SvJ, NOR/LtJ, A/J, C57BL/6, NOD/LtJ, DBA/2J, ALR/LtJ, C57L/J. Strains classified with ahl that DO NOT carry this mutation include: C3H/HeSnJ, I/LnJ,YBR/Ei, MRL/MpJ. [MGI Ref ID J:86905]
Allele Symbol		Hld
Allele Name		hippocampal lamination defect
Allele Type		Spontaneous
Strain of Origin		BALB/cJ
Gene Symbol and Name		Hld, hippocampal lamination defect
Chromosome		UN
General Note		Abnormal laminar organization of the pyramidal layer of the cerebellum, particularly in the proximal segment of the layer, occurs in the BALB/cJ strain. In normal strains, the latest formed or youngest neurons migrate past the earlier formed or older neurons to a position in the pyramidal layer that is superficial to that of the older cells. In BALB/cJ, the positions are reversed, with the older cells lying superior to the younger ones (J:5787). Since mossy fibers form synapses primarily with the older cells, this aberrant pattern of cell migration in BALB/c leads to a different pattern of mossy-fiber synapses, easily visualized with Timm's stain (J:5486). The dendritic excrescences induced by contact with mossy fibers on late-generated pyramidal cells in +/+ mice occur at sites on both the apical and basal dendrites; in Hld/Hld mice, they occur in two sites on the apical dendrites only (J:12029).
Gene Symbol and Name		Mdmg1, mandibular morphogenesis 1
Chromosome		17
Gene Common Name(s)		Mdmg-1;

Genotyping

Genotyping Information

Inbred mouse strains are maintained through sibling (sister x brother) matings; no genotyping required.

Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Bentvelzen P; Daams JH; Hageman P; Calafat J. 1970. Genetic transmission of viruses that incite mammary tumor in mice. Proc Natl Acad Sci U S A 67(1):377-84. [PubMed: 4318784]  [MGI Ref ID J:24803]

Champy MF; Selloum M; Zeitler V; Caradec C; Jung B; Rousseau S; Pouilly L; Sorg T; Auwerx J. 2008. Genetic background determines metabolic phenotypes in the mouse. Mamm Genome 19(5):318-31. [PubMed: 18392653]  [MGI Ref ID J:137669]

Ebbesen P. 1971. Reticulosarcoma and amyloid development in BALB/c mice inoculated with syngeneic cells from young and old donors. J Natl Cancer Inst 47(6):1241-5. [PubMed: 4941118]  [MGI Ref ID J:24297]

Heston WE; Vlahakis G. 1971. Mammary tumors, plaques, and hyperplastic alveolar nodules in various combinations of mouse inbred strains and the different lines of the mammary tumor virus. Int J Cancer 7(1):141-8. [PubMed: 4322934]  [MGI Ref ID J:24674]

Hinsdale ME; Kelly CL; Wood PA. 1993. Null allele at Bcd-1 locus in BALB/cByJ mice is due to a deletion in the short-chain acyl-CoA dehydrogenase gene and results in missplicing of mRNA. Genomics 16(3):605-11. [PubMed: 8325633]  [MGI Ref ID J:12776]

Petkov PM; Cassell MA; Sargent EE; Donnelly CJ; Robinson P; Crew V; Asquith S; Haar RV; Wiles MV. 2004. Development of a SNP genotyping panel for genetic monitoring of the laboratory mouse. Genomics 83(5):902-11. [PubMed: 15081119]  [MGI Ref ID J:89298]

Roderick TH; Langley SH; Leiter EH. 1985. Some unusual genetic characteristics of BALB/c and evidence for genetic variation among BALB/c substrains. Curr Top Microbiol Immunol 122:9-18. [PubMed: 3899524]  [MGI Ref ID J:24307]

Smith Richards BK; Belton BN; York B; Volaufova J. 2004. Mice bearing Acads mutation display altered postingestive but not 5-s orosensory response to dietary fat. Am J Physiol Regul Integr Comp Physiol 286(2):R311-9. [PubMed: 14592933]  [MGI Ref ID J:87779]

Sundberg JP; Hanson CA; Roop DR; Brown KS; Bedigian HG. 1991. Myoepitheliomas in inbred laboratory mice. Vet Pathol 28(4):313-23. [PubMed: 1719689]  [MGI Ref ID J:22767]

Wood PA; Amendt BA; Rhead WJ; Millington DS; Inoue F; Armstrong D. 1989. Short-chain acyl-coenzyme A dehydrogenase deficiency in mice. Pediatr Res 25(1):38-43. [PubMed: 2919115]  [MGI Ref ID J:14707]

Wood PA; Hinsdale ME; Kelly CL. 1993. Molecular detection of the Bcd-1 null allele in BALB/cByJ mice by polymerase chain reaction: a simple assay for genetic monitoring Mouse Genome 91(2):342-44.  [MGI Ref ID J:12812]

Additional References

Bouwknecht JA; Paylor R. 2002. Behavioral and physiological mouse assays for anxiety: a survey in nine mouse strains. Behav Brain Res 136(2):489-501. [PubMed: 12429412]  [MGI Ref ID J:80397]

Flaherty L; DiBiase K; Lynes MA; Seidman JG; Weinberger O; Rinchik EM. 1985. Characterization of a Q subregion gene in the murine major histocompatibility complex. Proc Natl Acad Sci U S A 82(5):1503-7. [PubMed: 2983348]  [MGI Ref ID J:7763]

International Nomenclature Committee. 1952. COMMITTEE on Standardized Nomenclature for Inbred Strains of Mice Cancer Res 12(8):602-13. [PubMed: 14945054]  [MGI Ref ID J:166288]

Moy SS; Nadler JJ; Young NB; Perez A; Holloway LP; Barbaro RP; Barbaro JR; Wilson LM; Threadgill DW; Lauder JM; Magnuson TR; Crawley JN. 2007. Mouse behavioral tasks relevant to autism: phenotypes of 10 inbred strains. Behav Brain Res 176(1):4-20. [PubMed: 16971002]  [MGI Ref ID J:138682]

Sass B; Peters RL; Kelloff GJ. 1976. Differences in tumor incidence in two substrains of Claude BALB/c (BALB/cfCd) mice, emphasizing renal, mammary, pancreatic, and synovial tumors. Lab Anim Sci 26(5):736-41. [PubMed: 185454]  [MGI Ref ID J:24705]

Smith BK; Andrews PK; West DB. 2000. Macronutrient diet selection in thirteen mouse strains. Am J Physiol Regul Integr Comp Physiol 278(4):R797-805. [PubMed: 10749765]  [MGI Ref ID J:61602]

Southwick CH; Clark LH. 1966. Agressive behavior and exploratory activity in fourteen mouse strains Am Zool 6:559.  [MGI Ref ID J:23455]

Teuscher C; Blankenhorn EP; Hickey WF. 1987. Differential susceptibility to actively induced experimental allergic encephalomyelitis and experimental allergic orchitis among BALB/c substrains. Cell Immunol 110(2):294-304. [PubMed: 2446778]  [MGI Ref ID J:149656]

Acads^del-J related

Amendt BA; Freneaux E; Reece C; Wood PA; Rhead WJ. 1992. Short-chain acyl-coenzyme A dehydrogenase activity, antigen, and biosynthesis are absent in the BALB/cByJ mouse. Pediatr Res 31(6):552-6. [PubMed: 1635815]  [MGI Ref ID J:1360]

Armstrong DL; Masiowski ML; Wood PA. 1993. Pathologic characterization of short-chain acyl-CoA dehydrogenase deficiency in BALB/cByJ mice. Am J Med Genet 47(6):884-92. [PubMed: 8279487]  [MGI Ref ID J:15293]

Guerra C; Koza RA; Walsh K; Kurtz DM; Wood PA; Kozak LP. 1998. Abnormal nonshivering thermogenesis in mice with inherited defects of fatty acid oxidation. J Clin Invest 102(9):1724-31. [PubMed: 9802886]  [MGI Ref ID J:50729]

Kruger C; Kumar KG; Mynatt RL; Volaufova J; Richards BK. 2012. Brain transcriptional responses to high-fat diet in Acads-deficient mice reveal energy sensing pathways. PLoS One 7(8):e41709. [PubMed: 22936979]  [MGI Ref ID J:191682]

Prochazka M; Leiter E. 1985. A null activity variant found at the butyryl CoA dehydrogenase (Bcd-1) locus in BALB/cByJ subline. Mouse News Lett 75:31.  [MGI Ref ID J:14101]

Qureshi IA; Leblanc D; Cyr D; Giguere R; Mitchell G. 1993. Breeding experiments to combine the X-linked sparse-fur (spf) mutation with the autosomal recessive BALB/cByJ strain: testing the biochemical phenotype of double-mutant mice as a model for ammonia: fatty acyl CoA synergism. Biochem Biophys Res Commun 191(2):744-9. [PubMed: 8461026]  [MGI Ref ID J:4165]

Reue K; Cohen RD. 1996. Acads gene deletion in BALB/cByJ mouse strain occurred after 1981 and is not present in BALB/cByJ-fld mutant mice. Mamm Genome 7(9):694-5. [PubMed: 8703125]  [MGI Ref ID J:35550]

Schiffer SP; Prochazka M; Jezyk PF; Roderick TH; Yudkoff M; Patterson DF. 1989. Organic aciduria and butyryl CoA dehydrogenase deficiency in BALB/cByJ mice. Biochem Genet 27(1-2):47-58. [PubMed: 2712823]  [MGI Ref ID J:9743]

Schuler AM; Gower BA; Matern D; Rinaldo P; Vockley J; Wood PA. 2005. Synergistic heterozygosity in mice with inherited enzyme deficiencies of mitochondrial fatty acid beta-oxidation. Mol Genet Metab 85(1):7-11. [PubMed: 15862275]  [MGI Ref ID J:98027]

Schuler AM; Gower BA; Matern D; Rinaldo P; Wood PA. 2004. Influence of dietary fatty acid chain-length on metabolic tolerance in mouse models of inherited defects in mitochondrial fatty acid beta-oxidation. Mol Genet Metab 83(4):322-9. [PubMed: 15589119]  [MGI Ref ID J:94661]

Skilling H; Coen PM; Fairfull L; Ferrell RE; Goodpaster BH; Vockley J; Goetzman ES. 2010. Brown adipose tissue function in short-chain acyl-CoA dehydrogenase deficient mice. Biochem Biophys Res Commun 400(3):318-22. [PubMed: 20727852]  [MGI Ref ID J:165594]

Su Z; Leduc MS; Korstanje R; Paigen B. 2010. Untangling HDL quantitative trait loci on mouse chromosome 5 and identifying Scarb1 and Acads as the underlying genes. J Lipid Res 51(9):2706-13. [PubMed: 20562441]  [MGI Ref ID J:164460]

Wood PA; Kelly-Kurtz CL; Hinsdale ME; Hamm DA; Rhead WJ. 1999. Lessons learned from the mouse model of short-chain acyl-CoA dehydrogenase deficiency Adv Exp Med Biol 466:395-402. [PubMed: 10709668]  [MGI Ref ID J:61299]

Ahr^b-2 related

Nebert DW; Considine N; Owens IS. 1973. Genetic expression of aryl hydrocarbon hydroxylase induction. VI. Control of other aromatic hydrocarbon-inducible mono-oxygenase activities at or near the same genetic locus. Arch Biochem Biophys 157(1):148-59. [PubMed: 4716952]  [MGI Ref ID J:84313]

Nebert DW; Gielen JE. 1972. Genetic regulation of aryl hydrocarbon hydroxylase induction in the mouse. Fed Proc 31(4):1315-25. [PubMed: 4114109]  [MGI Ref ID J:5282]

Nebert DW; Jensen NM; Shinozuka H; Kunz HW; Gill TJ 3rd. 1982. The Ah phenotype. Survey of forty-eight rat strains and twenty inbred mouse strains. Genetics 100(1):79-87. [PubMed: 7095422]  [MGI Ref ID J:6809]

Nebert DW; Robinson JR; Niwa A; Kumaki K; Poland AP. 1975. Genetic expression of aryl hydrocarbon hydroxylase activity in the mouse. J Cell Physiol 85(2 Pt 2 Suppl 1):393-414. [PubMed: 1091656]  [MGI Ref ID J:84317]

Niwa A; Kumaki K; Nebert DW; Poland AP. 1975. Genetic expression of aryl hydrocarbon hydroxylase activity in the mouse. Distinction between the 'responsive' homozygote and heterozygote at the Ah locus. Arch Biochem Biophys 166(2):559-64. [PubMed: 1119809]  [MGI Ref ID J:84316]

Poland A; Glover E. 1990. Characterization and strain distribution pattern of the murine Ah receptor specified by the Ahd and Ahb-3 alleles. Mol Pharmacol 38(3):306-12. [PubMed: 2169579]  [MGI Ref ID J:34840]

Poland A; Glover E; Taylor BA. 1987. The murine Ah locus: a new allele and mapping to chromosome 12. Mol Pharmacol 32(4):471-8. [PubMed: 2823093]  [MGI Ref ID J:8895]

Poland A; Palen D; Glover E. 1994. Analysis of the four alleles of the murine aryl hydrocarbon receptor. Mol Pharmacol 46(5):915-21. [PubMed: 7969080]  [MGI Ref ID J:22144]

Robinson JR; Considine N; Nebert DW. 1974. Genetic expression of aryl hydrocarbon hydroxylase induction. Evidence for the involvement of other genetic loci. J Biol Chem 249(18):5851-9. [PubMed: 4413562]  [MGI Ref ID J:84315]

Schmid FA; Pena RC; Robinson W; Tarnowski GS. 1967. Toxicity of intraperitoneal injections of 7, 12-dimethylbenz[a]anthracene in inbred mice. Cancer Res 27(3):558-62. [PubMed: 6021513]  [MGI Ref ID J:26440]

Schmidt JV; Carver LA; Bradfield CA. 1993. Molecular characterization of the murine Ahr gene. Organization, promoter analysis, and chromosomal assignment. J Biol Chem 268(29):22203-9. [PubMed: 8408082]  [MGI Ref ID J:15153]

Smith AG; Clothier B; Robinson S; Scullion MJ; Carthew P; Edwards R; Luo J; Lim CK; Toledano M. 1998. Interaction between iron metabolism and 2,3,7,8-tetrachlorodibenzo-p-dioxin in mice with variants of the Ahr gene: a hepatic oxidative mechanism. Mol Pharmacol 53(1):52-61. [PubMed: 9443932]  [MGI Ref ID J:45850]

Thomas PE; Hutton JJ; Taylor BA. 1973. Genetic relationship between aryl hydrocarbon hydroxylase inducibility and chemical carcinogen induced skin ulceration in mice. Genetics 74(4):655-9. [PubMed: 4750810]  [MGI Ref ID J:5387]

Cdh23^ahl related

Bosco A; Crish SD; Steele MR; Romero CO; Inman DM; Horner PJ; Calkins DJ; Vetter ML. 2012. Early reduction of microglia activation by irradiation in a model of chronic glaucoma. PLoS One 7(8):e43602. [PubMed: 22952717]  [MGI Ref ID J:191663]

Davis RR; Newlander JK; Ling X; Cortopassi GA; Krieg EF; Erway LC. 2001. Genetic basis for susceptibility to noise-induced hearing loss in mice. Hear Res 155(1-2):82-90. [PubMed: 11335078]  [MGI Ref ID J:69679]

Di Palma F; Pellegrino R; Noben-Trauth K. 2001. Genomic structure, alternative splice forms and normal and mutant alleles of cadherin 23 (Cdh23). Gene 281(1-2):31-41. [PubMed: 11750125]  [MGI Ref ID J:73941]

Fetoni AR; Picciotti PM; Paludetti G; Troiani D. 2011. Pathogenesis of presbycusis in animal models: a review. Exp Gerontol 46(6):413-25. [PubMed: 21211561]  [MGI Ref ID J:186964]

Han F; Yu H; Tian C; Chen HE; Benedict-Alderfer C; Zheng Y; Wang Q; Han X; Zheng QY. 2010. A new mouse mutant of the Cdh23 gene with early-onset hearing loss facilitates evaluation of otoprotection drugs. Pharmacogenomics J :. [PubMed: 20644563]  [MGI Ref ID J:174758]

Johnson KR; Erway LC; Cook SA; Willott JF; Zheng QY. 1997. A major gene affecting age-related hearing loss in C57BL/6J mice Hear Res 114(1-2):83-92. [PubMed: 9447922]  [MGI Ref ID J:44966]

Johnson KR; Longo-Guess C; Gagnon LH; Yu H; Zheng QY. 2008. A locus on distal chromosome 11 (ahl8) and its interaction with Cdh23 ahl underlie the early onset, age-related hearing loss of DBA/2J mice. Genomics 92(4):219-25. [PubMed: 18662770]  [MGI Ref ID J:139223]

Johnson KR; Yu H; Ding D; Jiang H; Gagnon LH; Salvi RJ. 2010. Separate and combined effects of Sod1 and Cdh23 mutations on age-related hearing loss and cochlear pathology in C57BL/6J mice. Hear Res 268(1-2):85-92. [PubMed: 20470874]  [MGI Ref ID J:163035]

Johnson KR; Zheng QY; Bykhovskaya Y; Spirina O; Fischel-Ghodsian N. 2001. A nuclear-mitochondrial DNA interaction affecting hearing impairment in mice. Nat Genet 27(2):191-4. [PubMed: 11175788]  [MGI Ref ID J:67312]

Johnson KR; Zheng QY; Noben-Trauth K. 2006. Strain background effects and genetic modifiers of hearing in mice. Brain Res 1091(1):79-88. [PubMed: 16579977]  [MGI Ref ID J:110459]

Johnson KR; Zheng QY; Weston MD; Ptacek LJ; Noben-Trauth K. 2005. The Mass1(frings) mutation underlies early onset hearing impairment in BUB/BnJ mice, a model for the auditory pathology of Usher syndrome IIC. Genomics 85(5):582-90. [PubMed: 15820310]  [MGI Ref ID J:97534]

Kane KL; Longo-Guess CM; Gagnon LH; Ding D; Salvi RJ; Johnson KR. 2012. Genetic background effects on age-related hearing loss associated with Cdh23 variants in mice. Hear Res 283(1-2):80-8. [PubMed: 22138310]  [MGI Ref ID J:183757]

Keithley EM; Canto C; Zheng QY; Fischel-Ghodsian N; Johnson KR. 2004. Age-related hearing loss and the ahl locus in mice. Hear Res 188(1-2):21-8. [PubMed: 14759567]  [MGI Ref ID J:87783]

Liu X; Bulgakov OV; Darrow KN; Pawlyk B; Adamian M; Liberman MC; Li T. 2007. Usherin is required for maintenance of retinal photoreceptors and normal development of cochlear hair cells. Proc Natl Acad Sci U S A 104(11):4413-8. [PubMed: 17360538]  [MGI Ref ID J:118927]

Manji SS; Williams LH; Miller KA; Ooms LM; Bahlo M; Mitchell CA; Dahl HH. 2011. A mutation in synaptojanin 2 causes progressive hearing loss in the ENU-mutagenised mouse strain Mozart. PLoS One 6(3):e17607. [PubMed: 21423608]  [MGI Ref ID J:171701]

Mathews CE; Leiter EH. 1999. Resistance of ALR/Lt islets to free radical-mediated diabetogenic stress is inherited as a dominant trait. Diabetes 48(11):2189-96. [PubMed: 10535453]  [MGI Ref ID J:109893]

Nadeau JH. 2003. Modifier genes and protective alleles in humans and mice. Curr Opin Genet Dev 13(3):290-5. [PubMed: 12787792]  [MGI Ref ID J:88012]

Noben-Trauth K; Latoche JR; Neely HR; Bennett B. 2010. Phenotype and genetics of progressive sensorineural hearing loss (Snhl1) in the LXS set of recombinant inbred strains of mice. PLoS One 5(7):e11459. [PubMed: 20628639]  [MGI Ref ID J:163117]

Noben-Trauth K; Zheng QY; Johnson KR. 2003. Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss. Nat Genet 35(1):21-3. [PubMed: 12910270]  [MGI Ref ID J:86905]

Noben-Trauth K; Zheng QY; Johnson KR; Nishina PM. 1997. mdfw: a deafness susceptibility locus that interacts with deaf waddler (dfw). Genomics 44(3):266-72. [PubMed: 9325047]  [MGI Ref ID J:38429]

Perrin BJ; Sonnemann KJ; Ervasti JM. 2010. beta-actin and gamma-actin are each dispensable for auditory hair cell development but required for Stereocilia maintenance. PLoS Genet 6(10):e1001158. [PubMed: 20976199]  [MGI Ref ID J:167543]

Vazquez AE; Jimenez AM; Martin GK; Luebke AE; Lonsbury-Martin BL. 2004. Evaluating cochlear function and the effects of noise exposure in the B6.CAST+Ahl mouse with distortion product otoacoustic emissions. Hear Res 194(1-2):87-96. [PubMed: 15276680]  [MGI Ref ID J:117746]

Zheng QY; Johnson KR. 2001. Hearing loss associated with the modifier of deaf waddler (mdfw) locus corresponds with age-related hearing loss in 12 inbred strains of mice. Hear Res 154(1-2):45-53. [PubMed: 11423214]  [MGI Ref ID J:70964]

Zheng QY; Scarborough JD; Zheng Y; Yu H; Choi D; Gillespie PG. 2012. Digenic inheritance of deafness caused by 8J allele of myosin-VIIA and mutations in other Usher I genes. Hum Mol Genet 21(11):2588-98. [PubMed: 22381527]  [MGI Ref ID J:183898]

Zilberstein Y; Liberman MC; Corfas G. 2012. Inner hair cells are not required for survival of spiral ganglion neurons in the adult cochlea. J Neurosci 32(2):405-10. [PubMed: 22238076]  [MGI Ref ID J:179911]

Hld related

Barber RP; Vaughn JE; Wimer RE; Wimer CC. 1974. Genetically-associated variations in the distribution of dentate granule cell synapses upon the pyramidal cell dendrites in mouse hippocampus. J Comp Neurol 156(4):417-34. [PubMed: 4137683]  [MGI Ref ID J:5486]

Nowakowski RS. 1984. Hippocampal lamination defect = Hld. Mouse News Lett 71:35.  [MGI Ref ID J:13989]

Nowakowski RS. 1984. The mode of inheritance of a defect in lamination in the hippocampus of BALB/c mice. J Neurogenet 1(3):249-58. [PubMed: 6536729]  [MGI Ref ID J:7947]

Nowakowski RS; Davis TL. 1985. Dendritic arbors and dendritic excrescences of abnormally positioned neurons in area CA3c of mice carrying the mutation hippocampal lamination defect. J Comp Neurol 239(3):267-75. [PubMed: 4044940]  [MGI Ref ID J:12029]

Vaughn JE; Matthews DA; Barber RP; Wimer CC; Wimer RE. 1977. Genetically-associated variations in the development of hippocampal pyramidal neurons may produce differences in mossy fiber connectivity. J Comp Neurol 173(1):41-51. [PubMed: 845286]  [MGI Ref ID J:5787]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX8
Room Number           MP14
Room Number           RB05

Colony Maintenance

Mating System	Sibling x Sibling         (Female x Male)   01-MAR-06
Breeding Considerations	This strain is a good breeder.
Diet Information	LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls

General Supply Notes

• Genomic DNA is available for this strain from the Mouse DNA Resource.

Important Note

This strain is homozygous for Cdh23^ahl, the age related hearing loss 1 mutation, which on this background results in progressive hearing loss with onset after 10 months of age.

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Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.

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The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX^® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX^® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX^® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.

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"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

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