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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Acadsdel-J
deletion, Jackson
MGI:3029768
Summary 5 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Acadsdel-J/Acadsdel-J BALB/cByJ MGI:3029782
cx2
Acadsdel-J/Acadsdel-J
Otcspf/Y
involves: BALB/cByJ * CD-1 MGI:3850512
cx3
Acadsdel-J/Acadsdel-J
Otcspf/Otc+
involves: BALB/cByJ * CD-1 MGI:3850513
cx4
Acadsdel-J/Acads+
Otcspf/Y
involves: BALB/cByJ * CD-1 MGI:3850518
cx5
Acadsdel-J/Acadsdel-J
Otcspf/Otcspf
involves: BALB/cByJ * CD-1 MGI:3850519


Genotype
MGI:3029782
hm1
Allelic
Composition
Acadsdel-J/Acadsdel-J
Genetic
Background
BALB/cByJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Acadsdel-J mutation (1 available); any Acads mutation (5 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• did not develop a preference for drinking water containing a corn oil emulsion (J:87779)
• did not develop a preference for drinking water containing a corn oil emulsion (J:87779)
• given a choice, selection of a fat/protein diet declines with time (J:87779)
• given a choice, selection of a fat/protein diet declines with time (J:87779)

homeostasis/metabolism
• block in short-chain fatty acid oxidation shown by reduced butyryl-CoA dehydrogenation in liver mitochondria (J:14707)
• block in short-chain fatty acid oxidation shown by reduced butyryl-CoA dehydrogenation in liver mitochondria (J:14707)
• 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 (J:50729)
• 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 (J:50729)
• serum glycine concentrations are markedly lower than controls (J:14707)
• serum glycine concentrations are markedly lower than controls (J:14707)
• slightly lower plasma carnitine level (J:14707)
• slightly lower plasma carnitine level (J:14707)
• hypoglycemia develops after 18 hours of fasting with mean serum glucose measuring less than half of control values (J:14707)
• hypoglycemia develops after 18 hours of fasting with mean serum glucose measuring less than half of control values (J:14707)
• HDL levels were significantly increased in mutant mice for both males and females fed either a regular chow or a high-fat diet (J:164460)
• HDL levels were significantly increased in mutant mice for both males and females fed either a regular chow or a high-fat diet (J:164460)
• ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher than in controls (J:4165)
• ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher than in controls (J:4165)
• organic aciduria at 7-14 weeks of age, caused by relatively large amounts of n-butyrylglycine and ethylmalonate in the urine (J:9743)
• organic aciduria at 7-14 weeks of age, caused by relatively large amounts of n-butyrylglycine and ethylmalonate in the urine (J:9743)
• 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 (J:14707)
• upon dosing with carnitine, mice excrete large amounts of butyryl-carnitine in the urine (J:14707)
• 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 (J:14707)
• upon dosing with carnitine, mice excrete large amounts of butyryl-carnitine in the urine (J:14707)

liver/biliary system
• fat deposits accumulate in the liver after 18 hours of fasting or with dietary fat challenge (J:14707)
• fat deposits accumulate in the liver after 18 hours of fasting or with dietary fat challenge (J:14707)

renal/urinary system
• ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher than in controls (J:4165)
• ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher than in controls (J:4165)
• organic aciduria at 7-14 weeks of age, caused by relatively large amounts of n-butyrylglycine and ethylmalonate in the urine (J:9743)
• organic aciduria at 7-14 weeks of age, caused by relatively large amounts of n-butyrylglycine and ethylmalonate in the urine (J:9743)
• 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 (J:14707)
• upon dosing with carnitine, mice excrete large amounts of butyryl-carnitine in the urine (J:14707)
• 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 (J:14707)
• upon dosing with carnitine, mice excrete large amounts of butyryl-carnitine in the urine (J:14707)

cellular
• block in short-chain fatty acid oxidation shown by reduced butyryl-CoA dehydrogenation in liver mitochondria (J:14707)
• block in short-chain fatty acid oxidation shown by reduced butyryl-CoA dehydrogenation in liver mitochondria (J:14707)

muscle
• wehn dosed with carnitine, mutant muscle exhibits a 9-fold increase in butyryl-carnitine concentration compared to controls (J:14707)
• wehn dosed with carnitine, mutant muscle exhibits a 9-fold increase in butyryl-carnitine concentration compared to controls (J:14707)

Mouse Models of Human Disease
OMIM ID Ref(s)
Acyl-Coa Dehydrogenase, Short-Chain, Deficiency Of; ACADSD 201470 J:9743




Genotype
MGI:3850512
cx2
Allelic
Composition
Acadsdel-J/Acadsdel-J
Otcspf/Y
Genetic
Background
involves: BALB/cByJ * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Acadsdel-J mutation (1 available); any Acads mutation (5 available)
Otcspf mutation (10 available); any Otc mutation (14 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 40% mortality in adults (J:4165)
• 40% mortality in adults (J:4165)

homeostasis/metabolism
• orotic acid, ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher in pre-weaning mutants than in wild-type controls (J:4165)
• however, levels of orotic acid are lower than in single mutant Otc males and in comparison to single homozygous Acads mutants, levels of ethylmalonate are lower, levels of methlysuccinate are no different, and levels of butyrylglycine and orotic acid are increased (J:4165)
• orotic acid, ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher in pre-weaning mutants than in wild-type controls (J:4165)
• however, levels of orotic acid are lower than in single mutant Otc males and in comparison to single homozygous Acads mutants, levels of ethylmalonate are lower, levels of methlysuccinate are no different, and levels of butyrylglycine and orotic acid are increased (J:4165)

renal/urinary system
• orotic acid, ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher in pre-weaning mutants than in wild-type controls (J:4165)
• however, levels of orotic acid are lower than in single mutant Otc males and in comparison to single homozygous Acads mutants, levels of ethylmalonate are lower, levels of methlysuccinate are no different, and levels of butyrylglycine and orotic acid are increased (J:4165)
• orotic acid, ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher in pre-weaning mutants than in wild-type controls (J:4165)
• however, levels of orotic acid are lower than in single mutant Otc males and in comparison to single homozygous Acads mutants, levels of ethylmalonate are lower, levels of methlysuccinate are no different, and levels of butyrylglycine and orotic acid are increased (J:4165)

integument
(J:4165)
(J:4165)




Genotype
MGI:3850513
cx3
Allelic
Composition
Acadsdel-J/Acadsdel-J
Otcspf/Otc+
Genetic
Background
involves: BALB/cByJ * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Acadsdel-J mutation (1 available); any Acads mutation (5 available)
Otcspf mutation (10 available); any Otc mutation (14 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• orotic acid, ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher in pre-weaning mutants than in wild-type controls (J:4165)
• however, levels of orotic acid are lower than in single mutant Otc males and in comparison to single homozygous Acads mutants, levels of ethylmalonate are lower, levels of methlysuccinate are no different, and levels of butyrylglycine and orotic acid are increased (J:4165)
• orotic acid, ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher in pre-weaning mutants than in wild-type controls (J:4165)
• however, levels of orotic acid are lower than in single mutant Otc males and in comparison to single homozygous Acads mutants, levels of ethylmalonate are lower, levels of methlysuccinate are no different, and levels of butyrylglycine and orotic acid are increased (J:4165)

renal/urinary system
• orotic acid, ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher in pre-weaning mutants than in wild-type controls (J:4165)
• however, levels of orotic acid are lower than in single mutant Otc males and in comparison to single homozygous Acads mutants, levels of ethylmalonate are lower, levels of methlysuccinate are no different, and levels of butyrylglycine and orotic acid are increased (J:4165)
• orotic acid, ethylmalonate, methylsuccinate, and butyrylglycine urinary excretion is higher in pre-weaning mutants than in wild-type controls (J:4165)
• however, levels of orotic acid are lower than in single mutant Otc males and in comparison to single homozygous Acads mutants, levels of ethylmalonate are lower, levels of methlysuccinate are no different, and levels of butyrylglycine and orotic acid are increased (J:4165)




Genotype
MGI:3850518
cx4
Allelic
Composition
Acadsdel-J/Acads+
Otcspf/Y
Genetic
Background
involves: BALB/cByJ * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Acadsdel-J mutation (1 available); any Acads mutation (5 available)
Otcspf mutation (10 available); any Otc mutation (14 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• butyrylglycine and orotic acid urinary excretion is increased compared to wild-type controls (J:4165)
• butyrylglycine and orotic acid urinary excretion is increased compared to wild-type controls (J:4165)
(J:4165)
(J:4165)

renal/urinary system
• butyrylglycine and orotic acid urinary excretion is increased compared to wild-type controls (J:4165)
• butyrylglycine and orotic acid urinary excretion is increased compared to wild-type controls (J:4165)
(J:4165)
(J:4165)




Genotype
MGI:3850519
cx5
Allelic
Composition
Acadsdel-J/Acadsdel-J
Otcspf/Otcspf
Genetic
Background
involves: BALB/cByJ * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Acadsdel-J mutation (1 available); any Acads mutation (5 available)
Otcspf mutation (10 available); any Otc mutation (14 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 40% mortality in adults (J:4165)
• 40% mortality in adults (J:4165)

homeostasis/metabolism
• orotic acid urinary excretion is increased (J:4165)
• orotic acid urinary excretion is increased (J:4165)

renal/urinary system
• orotic acid urinary excretion is increased (J:4165)
• orotic acid urinary excretion is increased (J:4165)





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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory