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Former Names NOD-Leprdb-5J/Lt (Changed: 07-MAR-07 ) Type Coisogenic; Mutant Strain; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse H2 Haplotype g7 Generation F105+4pN1 Donating Investigator Edward Leiter, The Jackson Laboratory Appearance
albino
Related Genotype: A/A Tyrc/TyrcDescription
Coisogenic, NOD/ShiLtJ-Leprdb-5J/LtJ (NOD- Leprdb-5J) homozygous mice develop juvenile onset obesity and type II diabetes coupled with suppression of spontaneous type I diabetes in which the NOD/ShiLtJ (NOD/ShiLt) strain is known. RT-PCR confirms the expression of the leptin receptor long isoform (Rb) in the hypothalamus. NOD/ShiLt mice homozygous for this mutation are viable and retain some fertility although the stock is maintained by heterozygous matings. By five weeks of age, homozygous mice are hyperphagic, eating twice the amount of mouse chow as lean controls and develop hyperglycemia not requiring insulin therapy for long tem survival (39+ weeks of age). Lean littermate mice (wild-type or heterozygotes) develop spontaneous type 1 diabetes in an age dependent (post-adolescent) manner. In contrast, Leprdb-5J homozygotes of both sexes develop hyperglycemia within one to two weeks of weaning. This is a type II diabetes based upon chronically elevated insulin and leptin levels maintained over periods up to one year. Pancreatic histopathology in most individuals shows islet hypertrophy with beta cell hyperplasia that is not restricted by insulitis. Insulitis, when present, is primarily peri-vascular/periductular and not intra-islet. Approximately one third of mutants progress to an end-stage Type II diabetes with decreases in body weight and plasma insulin whereas two thirds maintain obese body weight (35-45g) in association with a beta cell hypertrophy/hyperplasia phenotype (Lee et all., 2005). Originally, mutant males were reported as more severely affected than females, but current unpublished results show the same multiplicity of phenotypes in females. Serum lipid levels were not significantly increased in any of the Leprdb-5J homozygous mutant mice, current studies are showing an unexplained variability in this phenotype.
No significant pathology was originally observed in the liver or kidney (Lee et al., 2005). Splenic leukocytes from young, hyperglycemic mutants transferred into NOD.Rag1 recipients did not transfer type I diabetes over 13 weeks; whereas wild-type donor cells did. However, widespread insulitis was transferred by Leprdb-5J donors, indicating that their splenocytes contained a less-activated effector population at the time of transfer. Reciprocal bone marrow transfers confirmed that Leprdb-5J marrow contained diabetogenic stem cells; however, the slower diabetes generated by either wild-type or mutant marrow transferred into irradiated Leprdb-5J recipients indicated that the disturbed metabolic milieu produced by the obesity mutation was the major reason for suppressing the diabetogenic potential of marrow precursors (Lee et al., 2006).
Leptin has been reported to accelerate T1D onset in juvenile NOD females (Matarese et al., 2002). The leptin-resistant NOD-Leprdb-5J mouse is useful for dissecting the interaction between the endocrine and immune system in type 1 diabetes-prone mice; understanding the trophic factors generated to produce robust proliferation of B-cells; and studying the involvement of leptin signaling in cueing the cytopathic function of autoimmune T-effector cells.Development
The recessive Leprdb-5J mutation in the leptin receptor gene (Chromosome 4) arose spontaneously in the NOD/ShiLtJ (Stock No. 001976) production colony at The Jackson Laboratory in 2003 (Lee et al., 2005). The mutation was eliminated from breeder stock in the production colony but Dr. Edward Leiter retained heterozygous breeders. The Leprdb-5J mutation present in this NOD/ShiLt strain contains a G-T transversion mutation at position 640 encoding a valine instead of a glycine; eliminates a Hae III restriction site present in the wild-type receptor and resides in the distal portion of the extra-cellular domain (Lee et al., 2005). Thus, unlike the commonly studied Leprdb-1J mutation that lacks the intracellular signaling domain, the Leprdb-5J molecule retains an intact intracellular domain. In 2006, the T1DR received this strain at generation F105+4.
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 001976 NOD/ShiLtJ | ||
| Considerations for Choosing Controls | ||
NOD Strains
001976 NOD/ShiLtJ View NOD Strains (1 strain)
Strains carrying other alleles of Lepr
000709 129P3/J-Leprdb-3J/J 002048 B6 x C57BLKS-Dock7m Leprdb Myo15sh2-J/J 008518 B6.129-Leprtm1Mgmj/J 008320 B6.129-Leprtm2(cre)Rck/J 008385 B6.129-Leprtm2Mgmj/J 008327 B6.129P2-Leprtm1Rck/J 000697 B6.BKS(D)-Leprdb/J 000699 B6.Cg-Dock7m Leprdb/+ +/J 000642 BKS.Cg-Dock7m +/+ Leprdb/J 000700 BKS.Cg-Dock7m Leprdb/+ +/J 008340 BKS.Cg-Leprdb Nos3tm1Unc/RhrsJ 001192 BKS.Cg-meaJ Leprdb +/+ + Dock7m/J 000707 CBA.Cg-Dock7m Leprdb/+ +/J 006654 FVB.BKS(D)-Leprdb/ChuaJ 006846 STOCK Leprdb-9J/Jgn View Strains carrying other alleles of Lepr (15 strains)
View Mammalian Phenotype Terms
Mammalian Phenotype Terms
assigned by genotype
Leprdb-5J/Leprdb-5J
NOD/ShiLtJ
- digestive/alimentary phenotype
- degranulated pancreatic beta cells (MGI Ref ID J:87061)
- beta-degranulation in the islets of Langerhans
- pancreatic islet hyperplasia (MGI Ref ID J:87061)
- endocrine/exocrine gland phenotype
- degranulated pancreatic beta cells (MGI Ref ID J:87061)
- beta-degranulation in the islets of Langerhans
- pancreatic islet hyperplasia (MGI Ref ID J:87061)
- growth/size phenotype
- obese (MGI Ref ID J:87061)
- both males and females are obese by weaning age
- homeostasis/metabolism phenotype
- hyperglycemia (MGI Ref ID J:87061)
- age of onset by 5 weeks
- increased circulating insulin level (MGI Ref ID J:87061)
Leprdb-5J/Leprdb-5J
NOD/ShiLt-Leprdb-5J
- life span-post-weaning/aging
- premature death (MGI Ref ID J:118305)
- 3/6 males showing weight loss with severe hyperglycemia die by 41-49 weeks of age
- mice with juvenile-onset hyperglycemia did not require insulin therapy to survive to >39 weeks, whereas wild-type NOD littermates developed adult onset hyperglycemia, rapidly lost weight, and were euthanized
- growth/size phenotype
- increased weight gain (MGI Ref ID J:118305)
- within 1 week of weaning, mice gain 6-10 grams more than wild-type littermates
- females show rapid weight gain up ~15 weeks of age to a ~stable weight of 40 grams
- males also show marked weight gain after weaning; in 64.7% of males (11/17), postpubertal (from 7 weeks of age) weight gain continues
- weight loss (MGI Ref ID J:118305)
- 35.3% (6/17) homozygous males show a persistent decrease in weight beginning at 7-9 weeks and continuing to 39 weeks of age
- homeostasis/metabolism phenotype
- *normal* homeostasis/metabolism phenotype (MGI Ref ID J:118304)
- plasmacorticosterone levels in mutants and NOD controls are similar
- abnormal circulating glucose level (MGI Ref ID J:118305)
- hyperglycemia in all females and subset of males remits to normoglycemia with increasing age, while lean littermates show progression to type I diabetes
- 4/11 males showing maintained elevated body weight become normoglycemic (glucose 145 mg/dl at 39 weeks); remaining 7 males in this group are still hyperglycemic but exhibit lower mean blood glucose level (386 mg/dl) at 39 weeks
- hyperglycemia (MGI Ref ID J:118305)
- develops in most obese mice withing 2 weeks of weaning
- the males (6/17) showing decrease in body weight display severe hyperglycemia (glucose 581 mg/dl at 39 weeks)
- by 5-7 weeks of age, 4/5 females and 11/17 males are hyperglycemic (glucose >200 mg/dl) and most remain hyperglycemic through the peripubertal period (253-686 mg/dl at 13 weeks)
- increased circulating insulin level (MGI Ref ID J:118305)
- levels increase markedly above lean controls with age
- females remitting to normoglycemia still exhibit elevated insulin levels; males showing weight loss display reduced insulin levels (~nondiabetic control), while males maintaining elevated weight still show elevated levels
- increased circulating leptin level (MGI Ref ID J:118305)
- levels increase markedly above lean controls with age
- females remitting to normoglycemia still exhibit elevated leptin levels
- endocrine/exocrine gland phenotype
- abnormal pancreatic islet morphology (MGI Ref ID J:118305)
- animals that maintained high body weight with or without remission from intermediate levels of hyperglycemia have extremely hyperplastic islets with well-granulated beta cells; islets display peri-insulitis
- males exhibiting unrestrained hyperglycemia and weight loss have islets reduced in size and number of granulated beta cells, but showing minimal intraislet insulitis
- degranulated pancreatic beta cells (MGI Ref ID J:118305)
- islets are normal-sized with markedly degranulated beta cells
- pancreas inflammation (MGI Ref ID J:118305)
- perivascular/periductular infiltrates are present but nor intraislet infiltrates are observed
- periinsulitis (MGI Ref ID J:118305)
- digestive/alimentary phenotype
- abnormal pancreatic islet morphology (MGI Ref ID J:118305)
- animals that maintained high body weight with or without remission from intermediate levels of hyperglycemia have extremely hyperplastic islets with well-granulated beta cells; islets display peri-insulitis
- males exhibiting unrestrained hyperglycemia and weight loss have islets reduced in size and number of granulated beta cells, but showing minimal intraislet insulitis
- degranulated pancreatic beta cells (MGI Ref ID J:118305)
- islets are normal-sized with markedly degranulated beta cells
- pancreas inflammation (MGI Ref ID J:118305)
- perivascular/periductular infiltrates are present but nor intraislet infiltrates are observed
- periinsulitis (MGI Ref ID J:118305)
- behavior/neurological phenotype
- polyphagia (MGI Ref ID J:118305)
- early weight gain is associated with hyperphagia; at 5 weeks, mice consume twice as much as lean controls
- adipose tissue phenotype
- increased percent body fat (MGI Ref ID J:118305)
- at 15 weeks, females have 40.5% carcass fat vs 19.9% in lean littermates; males show 33% body fat vs 14.7% in lean males
- immune system phenotype
- abnormal CD4-positive T cell morphology (MGI Ref ID J:118304)
- CD4+ CD25+ T cells are slightly reduced in percentage compared to control NOD/LtJ mice (8.0 vs 9.5%)
- abnormal CD8-positive T cell morphology (MGI Ref ID J:118304)
- beta cell autoreactive CD8+ T cell clonotypes are decreased in mutants compared to NOD controls; IGRP and insulin-reactive CD8+ clonotypes are 5.7- and 1.7-fold lower in spleens of female mutants compared to controls
- pancreas inflammation (MGI Ref ID J:118305)
- perivascular/periductular infiltrates are present but nor intraislet infiltrates are observed
- periinsulitis (MGI Ref ID J:118305)
- hematopoietic system phenotype
- abnormal CD4-positive T cell morphology (MGI Ref ID J:118304)
- CD4+ CD25+ T cells are slightly reduced in percentage compared to control NOD/LtJ mice (8.0 vs 9.5%)
- abnormal CD8-positive T cell morphology (MGI Ref ID J:118304)
- beta cell autoreactive CD8+ T cell clonotypes are decreased in mutants compared to NOD controls; IGRP and insulin-reactive CD8+ clonotypes are 5.7- and 1.7-fold lower in spleens of female mutants compared to controls
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:
Leprdb-5J relatedDiabetes and Obesity Research
Hyperglycemia
Hyperinsulinemia
Diabetes and Obesity Research
Hyperglycemia
Hyperinsulinemia
| Allele Symbol | Leprdb-5J | ||
|---|---|---|---|
| Allele Name | diabetes 5 Jackson | ||
| Allele Type | Spontaneous | ||
| Mutation Made By | Edward Leiter, The Jackson Laboratory | ||
| Strain of Origin | NOD/ShiLtJ | ||
| Gene Symbol and Name | Lepr, leptin receptor | ||
| Chromosome | 4 | ||
| Gene Common Name(s) | CD295; Fa; LEPROT; Leprb; Modb1; OB-RGRP; OBR; db; diabetes; leptin receptor gene-related protein; obese-like; obl; | ||
| Molecular Note | A G to T transversion mutation was found in exon 13 effecting a glycine640valine change in the receptor. [MGI Ref ID J:87061] | ||
Genotyping Protocols
Leprdb-5J, Pyrosequencing
Leprdb-5J, Restriction Enzyme Digest
Helpful Links
Genotyping resources and troubleshooting
Lee CH; Chen YG; Chen J; Reifsnyder PC; Serreze DV; Clare-Salzler M; Rodriguez M; Wasserfall C; Atkinson MA; Leiter EH. 2006. Novel leptin receptor mutation in NOD/LtJ mice suppresses type 1 diabetes progression: II. Immunologic analysis. Diabetes 55(1):171-8. [PubMed: 16380490] [MGI Ref ID J:118304]
Lee CH; Reifsnyder PC; Naggert JK; Wasserfall C; Atkinson MA; Chen J; Leiter EH. 2005. Novel leptin receptor mutation in NOD/LtJ mice suppresses type 1 diabetes progression: I. Pathophysiological analysis. Diabetes 54(9):2525-32. [PubMed: 16123339] [MGI Ref ID J:118305]
Leiter EH; Lee C-H; Reifsnyder PC; Naggert JK. 2004. Leptin receptor deficiency in NOD/LtJ mice produces a type 2 diadetes syndrome with suppressed autoimmunity MGI Direct Data Submission :. [MGI Ref ID J:87061]
Colony Maintenance
Breeding & Husbandry Homozygotes of both sexes breed. However, homozygote x homozygote crosses are often unproductive.
| Pricing for USA, Canada and Mexico shipping destinations |
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Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
| Pricing for International shipping destinations |
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Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
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| Supply Notes |
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| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 001976 NOD/ShiLtJ | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| International - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
Purchasing Information
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| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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