MKS1 Chromosome 17

MKS transition zone complex subunit 1
219 variants 219 Health Risk

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What This Gene Does
The protein encoded by this gene localizes to the basal body and is required for formation of the primary cilium in ciliated epithelial cells. Mutations in this gene result in Meckel syndrome type 1 and in Bardet-Biedl syndrome type 13. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2009]
Gene Info
Gene Group
"B9 domain containing|MKS complex"
Locus Type
gene with protein product
Location
17q22
Ensembl
ENSG00000011143
Associated Conditions (35)
Joubert syndrome
Meckel-Gruber syndrome
Meckel syndrome
type 1
Bardet-Biedl syndrome 13
Joubert syndrome 28
MKS1-related disorder
Polydactyly
Nystagmus
Hypotonia
Bardet-Biedl syndrome
Inborn genetic diseases
Ovarian serous cystadenocarcinoma
Cervical cancer
Microcephaly
Optic atrophy
Retinal dystrophy
Spastic ataxia
Uterine corpus endometrial carcinoma
Sarcoma
+15 more conditions
Key Variants
RS111315726
Conflicting classifications of pathogenicity
Joubert syndrome, Meckel-Gruber syndrome, Meckel syndrome
Health Risk
RS11548967
Conflicting classifications of pathogenicity
Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
Health Risk
RS1156392394
Conflicting classifications of pathogenicity
Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
Health Risk
RS1244307754
Conflicting classifications of pathogenicity
Bardet-Biedl syndrome 13, Meckel syndrome, type 1
Health Risk
RS1291753612
Conflicting classifications of pathogenicity
Meckel-Gruber syndrome, Joubert syndrome, Bardet-Biedl syndrome 13
Health Risk
RS137853105
Conflicting classifications of pathogenicity
Bardet-Biedl syndrome 13, Meckel syndrome, type 1
Health Risk
RS142805406
Conflicting classifications of pathogenicity
Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
Health Risk
RS142813109
Conflicting classifications of pathogenicity
Bardet-Biedl syndrome 13, Meckel syndrome, type 1
Health Risk
RS1433060710
Conflicting classifications of pathogenicity
Joubert syndrome, Meckel-Gruber syndrome, Joubert syndrome
Health Risk
RS144635826
Conflicting classifications of pathogenicity
Bardet-Biedl syndrome 13, Meckel syndrome, type 1
Health Risk
RS1555596758
Conflicting classifications of pathogenicity
Meckel syndrome, type 1, Meckel-Gruber syndrome
Health Risk
RS185405908
Conflicting classifications of pathogenicity
Bardet-Biedl syndrome 13, Meckel syndrome, type 1
Health Risk
All Variants (219)
RSID Category Clinical Significance Conditions
RS62636631 Health Risk Conflicting classifications of pathogenicity Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
RS754404940 Health Risk Conflicting classifications of pathogenicity Meckel-Gruber syndrome, Joubert syndrome, MKS1-related disorder
RS754601373 Health Risk Conflicting classifications of pathogenicity Joubert syndrome, Meckel-Gruber syndrome, Inborn genetic diseases
RS756102768 Health Risk Conflicting classifications of pathogenicity Bardet-Biedl syndrome 13, Meckel syndrome, type 1
RS761061379 Health Risk Conflicting classifications of pathogenicity Bardet-Biedl syndrome 13, Meckel syndrome, type 1
RS762439479 Health Risk Conflicting classifications of pathogenicity Meckel syndrome, type 1, Bardet-Biedl syndrome 13
RS762989365 Health Risk Conflicting classifications of pathogenicity Meckel syndrome, type 1, Bardet-Biedl syndrome 13
RS763444295 Health Risk Conflicting classifications of pathogenicity Meckel-Gruber syndrome, Joubert syndrome, MKS1-related disorder
RS765026950 Health Risk Conflicting classifications of pathogenicity Joubert syndrome, Meckel-Gruber syndrome, Meckel syndrome
RS765242131 Health Risk Conflicting classifications of pathogenicity Meckel-Gruber syndrome, Joubert syndrome, Joubert syndrome 28
RS770493819 Health Risk Conflicting classifications of pathogenicity Meckel-Gruber syndrome, Joubert syndrome, Bardet-Biedl syndrome 13
RS772719574 Health Risk Conflicting classifications of pathogenicity Meckel syndrome, type 1, Bardet-Biedl syndrome 13
RS772852025 Health Risk Conflicting classifications of pathogenicity Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
RS773269657 Health Risk Conflicting classifications of pathogenicity Joubert syndrome, Meckel-Gruber syndrome, Joubert syndrome
RS775169308 Health Risk Conflicting classifications of pathogenicity Joubert syndrome, Meckel-Gruber syndrome, Meckel syndrome
RS778956664 Health Risk Conflicting classifications of pathogenicity Bardet-Biedl syndrome 13, Meckel syndrome, type 1
RS779953982 Health Risk Conflicting classifications of pathogenicity Joubert syndrome, Bardet-Biedl syndrome 13, Meckel syndrome
RS780161503 Health Risk Conflicting classifications of pathogenicity Meckel syndrome, type 1, Joubert syndrome 28
RS797045705 Health Risk Conflicting classifications of pathogenicity Joubert syndrome, Meckel-Gruber syndrome, MKS1-related disorder
RS886053171 Health Risk Conflicting classifications of pathogenicity Meckel syndrome, type 1, Bardet-Biedl syndrome 13
RS990955357 Health Risk Conflicting classifications of pathogenicity Meckel-Gruber syndrome, Joubert syndrome, Joubert syndrome 28
RS1214897554 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Bardet-Biedl syndrome 13
RS1269271727 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Bardet-Biedl syndrome 13
RS1273591389 Health Risk Likely pathogenic Meckel-Gruber syndrome, Joubert syndrome, Bardet-Biedl syndrome 13
RS12942386 Health Risk Likely pathogenic Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
RS1355211474 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Bardet-Biedl syndrome 13
RS1401015954 Health Risk Likely pathogenic Joubert syndrome, Meckel-Gruber syndrome, Joubert syndrome
RS1439561211 Health Risk Likely pathogenic Meckel syndrome, type 1, Meckel syndrome
RS1488635637 Health Risk Likely pathogenic Polydactyly, Nystagmus, Hypotonia
RS1555596845 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Meckel syndrome, type 1
RS1555596943 Health Risk Likely pathogenic Joubert syndrome 28, Meckel syndrome, type 1
RS1555601787 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Meckel syndrome, type 1
RS1567795088 Health Risk Likely pathogenic Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
RS1968523602 Health Risk Likely pathogenic Joubert syndrome, Meckel-Gruber syndrome, Joubert syndrome
RS1968931002 Health Risk Likely pathogenic Meckel-Gruber syndrome, Joubert syndrome, Meckel syndrome
RS1968973786 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Meckel syndrome, type 1
RS2143737350 Health Risk Likely pathogenic Meckel-Gruber syndrome, Joubert syndrome, Bardet-Biedl syndrome 13
RS2143742869 Health Risk Likely pathogenic Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
RS2143756871 Health Risk Likely pathogenic Meckel-Gruber syndrome, Joubert syndrome, Meckel-Gruber syndrome
RS2143805609 Health Risk Likely pathogenic Joubert syndrome, Meckel-Gruber syndrome, Bardet-Biedl syndrome 13
RS2509403348 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Bardet-Biedl syndrome 13
RS2509403404 Health Risk Likely pathogenic Joubert syndrome, Meckel-Gruber syndrome, Joubert syndrome
RS2509404204 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Meckel syndrome, type 1
RS2509407984 Health Risk Likely pathogenic Meckel-Gruber syndrome, Joubert syndrome, Bardet-Biedl syndrome 13
RS2509411772 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Bardet-Biedl syndrome 13
RS2509413057 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Bardet-Biedl syndrome 13
RS2509416449 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Bardet-Biedl syndrome 13
RS2509416760 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Meckel syndrome, type 1
RS2509416820 Health Risk Likely pathogenic Bardet-Biedl syndrome 13, Bardet-Biedl syndrome 13
RS2509426093 Health Risk Likely pathogenic Meckel syndrome, type 1, Joubert syndrome 28
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