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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Ugcgtm1Hjg
targeted mutation 1, Hermann-Josef Grone
MGI:3603453
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
 		Gal3st1tm1.1Hjg/Gal3st1tm1.1Hjg
Ugcgtm1Hjg/Ugcgtm1Hjg
Pax8tm1.1(cre)Mbu/Pax8+ 		involves: 129P2/OlaHsd * C57BL/6 MGI:5521180
cn2
 		Ugcgtm1Hjg/Ugcgtm1Hjg
Pax8tm1.1(cre)Mbu/Pax8+ 		involves: 129P2/OlaHsd * C57BL/6 MGI:5521181
cn3
 		Ugcgtm1Hjg/Ugcgtm1.1Hjg
Tg(Nes-cre)1Kln/0 		involves: 129P2/OlaHsd * C57BL/6 * SJL MGI:3604768


Genotype
MGI:5521180
cn1
Allelic
Composition		 Gal3st1tm1.1Hjg/Gal3st1tm1.1Hjg
Ugcgtm1Hjg/Ugcgtm1Hjg
Pax8tm1.1(cre)Mbu/Pax8+
Genetic
Background		 involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gal3st1tm1.1Hjg mutation (0 available); any Gal3st1 mutation (20 available)
Pax8tm1.1(cre)Mbu mutation (1 available); any Pax8 mutation (32 available)
Ugcgtm1Hjg mutation (0 available); any Ugcg mutation (89 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
mortality/aging ( J:197393 )
N
• normal life span

renal/urinary system
renal/urinary system phenotype ( J:197393 )
N
• normal kidney morphology and creatinine clearance
decreased urine ammonia level ( J:197393 )
• decreased ammonium excretion
• reduced ability to increase urinary ammonia
decreased urine pH ( J:197393 )
• higher excretion of titratable acid relative to controls
• less ability to recover from high acid in the diet
abnormal kidney papilla morphology ( J:197393 )
• ammonium content of the renal papilla is reduced 30% compared to controls

homeostasis/metabolism
homeostasis/metabolism phenotype ( J:197393 )
N
• normal blood pH
• normal blood bicarbonate levels
decreased urine ammonia level ( J:197393 )
• decreased ammonium excretion
• reduced ability to increase urinary ammonia
decreased urine pH ( J:197393 )
• higher excretion of titratable acid relative to controls
• less ability to recover from high acid in the diet




Genotype
MGI:5521181
cn2
Allelic
Composition		 Ugcgtm1Hjg/Ugcgtm1Hjg
Pax8tm1.1(cre)Mbu/Pax8+
Genetic
Background		 involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pax8tm1.1(cre)Mbu mutation (1 available); any Pax8 mutation (32 available)
Ugcgtm1Hjg mutation (0 available); any Ugcg mutation (89 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
mortality/aging ( J:197393 )
N
• normal life span

renal/urinary system
renal/urinary system phenotype ( J:197393 )
N
• normal kidney morphology and creatinine clearance

homeostasis/metabolism




Genotype
MGI:3604768
cn3
Allelic
Composition		 Ugcgtm1Hjg/Ugcgtm1.1Hjg
Tg(Nes-cre)1Kln/0
Genetic
Background		 involves: 129P2/OlaHsd * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Nes-cre)1Kln mutation (4 available)
Ugcgtm1.1Hjg mutation (0 available); any Ugcg mutation (89 available)
Ugcgtm1Hjg mutation (0 available); any Ugcg mutation (89 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
postnatal lethality, complete penetrance ( J:101155 )
• all mutants die by 24 days of age with a median age of lethality of 18 days

homeostasis/metabolism
abnormal lipid homeostasis ( J:101155 )
• ceramide concentration in the brain is decreased about 1.7-fold at P0 and P15, but sphingomyelin levels are consistently increased after birth

behavior/neurological
impaired righting response ( J:101155 )
• when placed on their ridge mutants take about 6 seconds to roll over compared to less than 0.5 seconds for control littermates
ataxia ( J:101155 )
• mutants develop severe ataxia with a shuffling gait and strong equilibrium impairments
impaired balance ( J:101155 )
• mutants are unable to balance on a rotating rod
abnormal gait ( J:101155 )
• shuffling gait

nervous system
abnormal Purkinje cell morphology ( J:101155 )
• the dendritic tree shows decreased height and arborization in mutant cerebella
• in culture neurite length and the number of branching points are decreased for Purkinje cells from E15.5 mutant embryos
abnormal axon morphology ( J:101155 )
• surface area of the axon of the femoral nerve is increased
abnormal myelination ( J:101155 )
• myelin surface area of the femoral nerve is increased and in some mutants splitting of the myelin sheath is seen

growth/size/body
decreased body weight ( J:101155 )
• body weight is similar to control littermates at birth, but after birth mutants gain less weight than their control littermates




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Send questions and comments to User Support. 		last database update
09/30/2025
MGI 6.24 		The Jackson Laboratory