All Phenotypes Por<tm1Wolf> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Atp8b1^tm1Nbf/Atp8b1^tm1Nbf Por^tm1Wolf/Por^tm1Wolf Speer6-ps1^{Tg(Alb-cre)21Mgn}/Speer6-ps1⁺	involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6 * DBA	MGI:5618808
cn2	Por^tm1Wolf/Por^tm1Wolf Speer6-ps1^{Tg(Alb-cre)21Mgn}/Speer6-ps1⁺	involves: 129P2/OlaHsd * C57BL/6 * DBA/2	MGI:2656922
cn3	Por^tm2Wolf/Por^tm1Wolf Speer6-ps1^{Tg(Alb-cre)21Mgn}/Speer6-ps1⁺	involves: 129P2/OlaHsd * C57BL/6 * DBA/2	MGI:2656921
cn4	Abcb4^tm1Bor/Abcb4^tm1Bor Por^tm1Wolf/Por^tm1Wolf Speer6-ps1^{Tg(Alb-cre)21Mgn}/Speer6-ps1⁺	involves: 129P2/OlaHsd * C57BL/6 * DBA * FVB/N	MGI:5618809

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

increased susceptibility to induced morbidity/mortality ( J:215915 )

• mice fed a 0.03% cholic acid-supplemented diet directly from weaning die within a week after the start of the diet

• however, mice fed a 0.03% cholic acid-supplemented diet starting at 4 weeks of age for 4 weeks survive

growth/size/body

weight loss ( J:215915 )

• mice fail to gain weight and even lose weight after starting of 0.03% cholic acid-supplemented diet at 4 weeks of age

increased liver weight ( J:215915 )

• liver weights are increased in mice fed a cholic acid-supplemented diet for 4 weeks starting at 4 weeks of age

• mice fed a cholic acid-supplemented diet for 4 weeks starting at 4 weeks of age do not exhibit fibrosis in the liver

homeostasis/metabolism

increased circulating bilirubin level ( J:215915 )

• after 4 weeks of cholic acid-supplemented diet started at 4 weeks of age, mice exhibit enhanced plasma bilirubin levels

increased circulating alanine transaminase level ( J:215915 )

• after 4 weeks of cholic acid-supplemented diet started at 4 weeks of age, mice exhibit a mild elevation in plasma alanine transaminase levels

increased circulating alkaline phosphatase level ( J:215915 )

• after 4 weeks of cholic acid-supplemented diet started at 4 weeks of age, mice exhibit a mild elevation in plasma alkaline phosphatase levels

abnormal bile salt homeostasis ( J:215915 )

• after 4 weeks on a cholic acid-supplemented diet, plasma bile salts predominantly consist of about 90% taurocholate (TC) and about 7% tauro-beta-muricholate (TbetaMC) and a low concentration of about 0.4% of the secondary bile salt taurochenodeoxycholate (TDC) is seen compared to about 70% TC and 20% TbetaMC in single Atp8b1 homozygotes

• after 4 weeks on a cholic acid-supplemented diet, biliary bile salts predominantly consist of about 90% TC and about 9% TbetaMC compared to 55% TC and 40% TbetaMC in single Atp8b1 homozygotes

• after 4 weeks on a cholic acid-supplemented diet, bile salt hydrophobicity index of plasma is elevated but the hydrophobicity index of bile is unaltered, indicating that the plasma bile salt pool is more hydrophobic

increased bile salt level ( J:215915 )

• after 4 weeks of cholic acid-supplemented diet started at 4 weeks of age, mice exhibit a strong elevation in plasma bile salt levels

liver/biliary system

bile duct proliferation ( J:215915 )

• mice fed a cholic acid-supplemented diet for 4 weeks starting at 4 weeks of age, exhibit a slight increase in bile duct proliferation

increased liver weight ( J:215915 )

• liver weights are increased in mice fed a cholic acid-supplemented diet for 4 weeks starting at 4 weeks of age

• mice fed a cholic acid-supplemented diet for 4 weeks starting at 4 weeks of age do not exhibit fibrosis in the liver

abnormal bile secretion ( J:215915 )

• after 4 weeks of cholic acid-supplemented diet started at 4 weeks of age, bile flow is strongly (about 3-fold) reduced

• mice, however show normal bile salt output when fed a cholic acid-supplemented diet for 4 weeks starting at 4 weeks of age

cholestasis ( J:215915 )

• mice fed a cholic acid-supplemented diet for 4 weeks starting at 4 weeks of age develop a severe cholestatic phenotype with mild liver damage

endocrine/exocrine glands

bile duct proliferation ( J:215915 )

• mice fed a cholic acid-supplemented diet for 4 weeks starting at 4 weeks of age, exhibit a slight increase in bile duct proliferation

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
intrahepatic cholestasis		DOID:1852		J:215915

Allelic Composition	Por^tm1Wolf/Por^tm1Wolf Speer6-ps1^{Tg(Alb-cre)21Mgn}/Speer6-ps1⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Por^tm1Wolf mutation (2 available); any Por mutation (78 available)
Speer6-ps1^{Tg(Alb-cre)21Mgn} mutation (6 available); any Speer6-ps1 mutation (4 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

growth/size/body

enlarged liver ( J:82951 )

• liver almost doubled in size

homeostasis/metabolism

decreased circulating cholesterol level ( J:82951 )

• 65% reduction in serum cholesterol level

decreased circulating triglyceride level ( J:82951 )

• 50% reduction in serum triglyceride level

increased circulating alanine transaminase level ( J:82951 )

abnormal bile salt level ( J:82951 )

• 90% reduction in bile acid volume in the gall bladder

abnormal xenobiotic pharmacokinetics ( J:82951 )

• abnormal metabolism of acetaminophen and pentobarbital

abnormal acetaminophen metabolism ( J:82951 )

liver/biliary system

enlarged liver ( J:82951 )

• liver almost doubled in size

macrovesicular hepatic steatosis ( J:82951 )

• macrovesicular fatty changes

microvesicular hepatic steatosis ( J:82951 )

• microvesicular fatty changes

pale liver ( J:82951 )

reproductive system

reduced fertility ( J:82951 )

• reduced fertility when mated with other null animals

decreased litter size ( J:82951 )

• reduced litter size when mated with other null animals

Allelic Composition	Por^tm2Wolf/Por^tm1Wolf Speer6-ps1^{Tg(Alb-cre)21Mgn}/Speer6-ps1⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Por^tm1Wolf mutation (2 available); any Por mutation (78 available)
Por^tm2Wolf mutation (0 available); any Por mutation (78 available)
Speer6-ps1^{Tg(Alb-cre)21Mgn} mutation (6 available); any Speer6-ps1 mutation (4 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

growth/size/body

enlarged liver ( J:82951 )

• liver almost doubled in size

homeostasis/metabolism

decreased circulating cholesterol level ( J:82951 )

• 65% reduction in serum cholesterol

decreased circulating triglyceride level ( J:82951 )

• 50% reduction in serum triglyceride level

increased circulating alanine transaminase level ( J:82951 )

abnormal bile salt level ( J:82951 )

• 90% reduction in bile acid volume in the gall bladder

abnormal xenobiotic pharmacokinetics ( J:82951 )

• altered metabolism of pentobarbital and acetominophen

abnormal acetaminophen metabolism ( J:82951 )

liver/biliary system

enlarged liver ( J:82951 )

• liver almost doubled in size

macrovesicular hepatic steatosis ( J:82951 )

• macrovesicular fatty changes

microvesicular hepatic steatosis ( J:82951 )

• microvesicular fatty changes

pale liver ( J:82951 )

reproductive system

reduced fertility ( J:82951 )

• reduced fertility when mated with other null animals

decreased litter size ( J:82951 )

• reduced litter size when mated with other null animals

Allelic Composition	Abcb4^tm1Bor/Abcb4^tm1Bor Por^tm1Wolf/Por^tm1Wolf Speer6-ps1^{Tg(Alb-cre)21Mgn}/Speer6-ps1⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * DBA * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Abcb4^tm1Bor mutation (1 available); any Abcb4 mutation (62 available)
Por^tm1Wolf mutation (2 available); any Por mutation (78 available)
Speer6-ps1^{Tg(Alb-cre)21Mgn} mutation (6 available); any Speer6-ps1 mutation (4 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

endocrine/exocrine glands

bile duct proliferation ( J:215915 )

• mice fed a cholic acid-supplemented diet for 8 weeks exhibit bile duct proliferation

growth/size/body

growth/size/body region phenotype ( J:215915 )

N	• mice fed a 0.03% cholic acid-supplemented diet directly from weaning exhibit normal growth

increased liver weight ( J:215915 )

• mice fed a cholic acid-supplemented diet for 8 weeks exhibit an increased liver to body weight ratio

cellular

increased hepatocyte proliferation ( J:215915 )

• mice fed a cholic acid-supplemented diet for 8 weeks exhibit increased hepatocyte proliferation

homeostasis/metabolism

increased circulating bilirubin level ( J:215915 )

• plasma bilirubin levels are mildly increased at weaning and are further increased after 8 weeks of 0.03% cholic acid-supplemented diet

increased circulating alanine transaminase level ( J:215915 )

• plasma alanine transaminase levels are strongly elevated after 8 weeks of cholic acid-supplemented diet

increased circulating alkaline phosphatase level ( J:215915 )

• plasma alkaline phosphatase levels are strongly elevated after 8 weeks of cholic acid-supplemented diet

abnormal bile salt homeostasis ( J:215915 )

• on a cholic acid-supplemented diet, plasma bile salts predominantly consist of about 90% taurocholate (TC) and about 7% tauro-beta-muricholate (TbetaMC) compared to about 60% TC and 23% TbetaMC in single Abcb4 homozygotes

• on a cholic acid-supplemented diet, biliary bile salts predominantly consist of about 90% TC and about 2.5% of the secondary bile salt taurochenodeoxycholate (TDC) compared to 75% TC and 20% TbetaMC in single Abcb4 homozygotes

• on a cholic acid-supplemented diet, the biliary hydrophobicity index, but not the plasma hydrophobicity index, is elevated, indicating a more hydrophobic biliary bile salt pool

• however, mice fed a cholic acid-supplemented diet for 8 weeks exhibit normal bile flow and biliary bile salt output

increased bile salt level ( J:215915 )

• plasma bile salt levels are strongly elevated after 8 weeks of cholic acid-supplemented diet

liver/biliary system

increased hepatocyte proliferation ( J:215915 )

• mice fed a cholic acid-supplemented diet for 8 weeks exhibit increased hepatocyte proliferation

bile duct proliferation ( J:215915 )

• mice fed a cholic acid-supplemented diet for 8 weeks exhibit bile duct proliferation

increased liver weight ( J:215915 )

• mice fed a cholic acid-supplemented diet for 8 weeks exhibit an increased liver to body weight ratio

liver fibrosis ( J:215915 )

• mice fed a regular diet exhibit portal fibrosis

• mice fed a cholic acid-supplemented diet for 8 weeks exhibit portal fibrosis that is more severe than in single Abcb4 homozygotes

cholestasis ( J:215915 )

• mice fed a cholic acid-supplemented diet for 8 weeks develop a severe cholestatic phenotype with severe liver damage

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
intrahepatic cholestasis		DOID:1852		J:215915

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