BBS5 (Bardet-Biedl syndrome 5)

2014-11-01  

Identity

HGNC
LOCATION
2q31.1
LOCUSID
ALIAS
-
FUSION GENES

Other Information

Locus ID:

NCBI: 129880
MIM: 603650
HGNC: 970
Ensembl: ENSG00000163093

Variants:

dbSNP: 129880
ClinVar: 129880
TCGA: ENSG00000163093
COSMIC: BBS5

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000163093ENST00000295240Q8N3I7
ENSG00000163093ENST00000295240A0A0S2Z626
ENSG00000163093ENST00000392663Q8N3I7
ENSG00000163093ENST00000392663A0A0S2Z6S7
ENSG00000163093ENST00000443151F8WBR7

Expression (GTEx)

0
5
10
15
20
25
30
35

Pathways

PathwaySourceExternal ID
Organelle biogenesis and maintenanceREACTOMER-HSA-1852241
Cilium AssemblyREACTOMER-HSA-5617833
Cargo trafficking to the periciliary membraneREACTOMER-HSA-5620920
BBSome-mediated cargo-targeting to ciliumREACTOMER-HSA-5620922

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
335604202021A mouse model of BBS identifies developmental and homeostatic effects of BBS5 mutation and identifies novel pituitary abnormalities.6
335728602021BBS Proteins Affect Ciliogenesis and Are Essential for Hedgehog Signaling, but Not for Formation of iPSC-Derived RPE-65 Expressing RPE-Like Cells.8
335604202021A mouse model of BBS identifies developmental and homeostatic effects of BBS5 mutation and identifies novel pituitary abnormalities.6
335728602021BBS Proteins Affect Ciliogenesis and Are Essential for Hedgehog Signaling, but Not for Formation of iPSC-Derived RPE-65 Expressing RPE-Like Cells.8
328112492020Novel compound heterozygous pathogenic BBS5 variants in Filipino siblings with Bardet-Biedl syndrome (BBS).3
328112492020Novel compound heterozygous pathogenic BBS5 variants in Filipino siblings with Bardet-Biedl syndrome (BBS).3
308503972019Novel splicing variant c. 208+2T>C in BBS5 segregates with Bardet-Biedl syndrome in an Iranian family by targeted exome sequencing.10
308503972019Novel splicing variant c. 208+2T>C in BBS5 segregates with Bardet-Biedl syndrome in an Iranian family by targeted exome sequencing.10
268670082016A Splice Variant of Bardet-Biedl Syndrome 5 (BBS5) Protein that Is Selectively Expressed in Retina.5
275205852016Expression of CXCL6 and BBS5 that may be glaucoma relevant genes is regulated by PITX2.4
268670082016A Splice Variant of Bardet-Biedl Syndrome 5 (BBS5) Protein that Is Selectively Expressed in Retina.5
275205852016Expression of CXCL6 and BBS5 that may be glaucoma relevant genes is regulated by PITX2.4
208015162011Simultaneous mutation detection in 90 retinal disease genes in multiple patients using a custom-designed 300-kb retinal resequencing chip.12
208015162011Simultaneous mutation detection in 90 retinal disease genes in multiple patients using a custom-designed 300-kb retinal resequencing chip.12
190774382009Analysis of 30 genes (355 SNPS) related to energy homeostasis for association with adiposity in European-American and Yup'ik Eskimo populations.12

Citation

Dessen P

BBS5 (Bardet-Biedl syndrome 5)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/60719/bbs5-(bardet-biedl-syndrome-5)