GBA1 Chromosome 1

Glucosylceramidase beta 1
180 variants 180 Health Risk

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What This Gene Does
This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2010]
Gene Info
Gene Group
"Glycoside hydrolase family|Glucosylceramidases"
Locus Type
gene with protein product
Location
1q22
Ensembl
ENSG00000177628
Associated Conditions (36)
7 conditions
Parkinson disease
late-onset
Gaucher disease
Gaucher disease type I
Gaucher disease-ophthalmoplegia-cardiovascular calcification syndrome
Gaucher disease type II
Gaucher disease type III
Alpha thalassemia-X-linked intellectual disability syndrome
Colon adenocarcinoma
Inborn genetic diseases
GBA1-related disorder
Gaucher disease perinatal lethal
Lewy body dementia
Parkinsonian disorder
Rigidity
Cogwheel rigidity
Tremor
6 conditions
See cases
+16 more conditions
Key Variants
RS1057942
Conflicting classifications of pathogenicity
7 conditions, Parkinson disease, late-onset
Health Risk
RS1141802
Conflicting classifications of pathogenicity
Gaucher disease type I, Gaucher disease type I
Health Risk
RS121908297
Conflicting classifications of pathogenicity
Gaucher disease type I, Gaucher disease type I
Health Risk
RS121908301
Conflicting classifications of pathogenicity
Gaucher disease type I, Gaucher disease type I
Health Risk
RS1264734195
Conflicting classifications of pathogenicity
Gaucher disease-ophthalmoplegia-cardiovascular calcification syndrome, Gaucher disease type I, Gaucher disease type II
Health Risk
RS1296507371
Conflicting classifications of pathogenicity
Gaucher disease type I, 7 conditions, Gaucher disease type I
Health Risk
RS1376479747
Conflicting classifications of pathogenicity
Alpha thalassemia-X-linked intellectual disability syndrome, Alpha thalassemia-X-linked intellectual disability syndrome
Health Risk
RS138498426
Conflicting classifications of pathogenicity
Gaucher disease type I, Gaucher disease type II, Gaucher disease
Health Risk
RS1392291885
Conflicting classifications of pathogenicity
Gaucher disease type I, Gaucher disease, Gaucher disease type I
Health Risk
RS145773486
Conflicting classifications of pathogenicity
Gaucher disease type I, Gaucher disease type I
Health Risk
RS147411159
Conflicting classifications of pathogenicity
Gaucher disease, Gaucher disease
Health Risk
RS1484043383
Conflicting classifications of pathogenicity
Gaucher disease, Gaucher disease
Health Risk
All Variants (180)
RSID Category Clinical Significance Conditions
RS1057942 Health Risk Conflicting classifications of pathogenicity 7 conditions, Parkinson disease, late-onset
RS1141802 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type I
RS121908297 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type I
RS121908301 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type I
RS1264734195 Health Risk Conflicting classifications of pathogenicity Gaucher disease-ophthalmoplegia-cardiovascular calcification syndrome, Gaucher disease type I, Gaucher disease type II
RS1296507371 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, 7 conditions, Gaucher disease type I
RS1376479747 Health Risk Conflicting classifications of pathogenicity Alpha thalassemia-X-linked intellectual disability syndrome, Alpha thalassemia-X-linked intellectual disability syndrome
RS138498426 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type II, Gaucher disease
RS1392291885 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease, Gaucher disease type I
RS145773486 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type I
RS147411159 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Gaucher disease
RS1484043383 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Gaucher disease
RS1671660634 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS1671865905 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Gaucher disease
RS188978150 Health Risk Conflicting classifications of pathogenicity GBA1-related disorder, Gaucher disease, GBA1-related disorder
RS199928507 Health Risk Conflicting classifications of pathogenicity Gaucher disease type II, Gaucher disease type I, Gaucher disease type III
RS2148075570 Health Risk Conflicting classifications of pathogenicity
RS2524839442 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type I
RS368060 Health Risk Conflicting classifications of pathogenicity
RS368832292 Health Risk Conflicting classifications of pathogenicity
RS369068553 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type I
RS374117599 Health Risk Conflicting classifications of pathogenicity
RS374306700 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease-ophthalmoplegia-cardiovascular calcification syndrome, Gaucher disease type II
RS377143075 Health Risk Conflicting classifications of pathogenicity
RS377325220 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease, Gaucher disease type I
RS398123526 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Gaucher disease
RS398123531 Health Risk Conflicting classifications of pathogenicity
RS539166948 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Lewy body dementia, Gaucher disease type I
RS61748906 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type II, Gaucher disease type III
RS74731340 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type II, Gaucher disease type III
RS749227753 Health Risk Conflicting classifications of pathogenicity
RS75548401 Health Risk Conflicting classifications of pathogenicity Parkinson disease, late-onset, Gaucher disease perinatal lethal
RS755512507 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Gaucher disease
RS75671029 Health Risk Conflicting classifications of pathogenicity Parkinson disease, late-onset, Gaucher disease
RS758447515 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Gaucher disease type I, Gaucher disease
RS765633380 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease-ophthalmoplegia-cardiovascular calcification syndrome, Gaucher disease type II
RS770796008 Health Risk Conflicting classifications of pathogenicity Gaucher disease type I, Gaucher disease type II, Gaucher disease type III
RS781152868 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Gaucher disease
RS781306264 Health Risk Conflicting classifications of pathogenicity
RS786200979 Health Risk Conflicting classifications of pathogenicity
RS794727708 Health Risk Conflicting classifications of pathogenicity Gaucher disease, Gaucher disease
RS80222298 Health Risk Conflicting classifications of pathogenicity
RS80356763 Health Risk Conflicting classifications of pathogenicity Gaucher disease perinatal lethal, Gaucher disease, Gaucher disease type I
RS367968666 Health Risk Conflicting classifications of pathogenicity; other Gaucher disease-ophthalmoplegia-cardiovascular calcification syndrome, Gaucher disease type I, Gaucher disease type II
RS2230288 Health Risk Conflicting classifications of pathogenicity; risk factor Parkinsonian disorder, Rigidity, Cogwheel rigidity
RS1057524702 Health Risk Likely pathogenic
RS1161552095 Health Risk Likely pathogenic GBA1-related disorder, GBA1-related disorder
RS1178732315 Health Risk Likely pathogenic Gaucher disease, Gaucher disease
RS121908305 Health Risk Likely pathogenic Gaucher disease type II, Gaucher disease, Gaucher disease perinatal lethal
RS121908307 Health Risk Likely pathogenic Gaucher disease type I, Gaucher disease type I
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