BBS9 (Bardet-Biedl syndrome 9)

2007-02-01  

Identity

HGNC
LOCATION
7p14.3
LOCUSID
ALIAS
B1,C18,D1,PTHB1
FUSION GENES

Other Information

Locus ID:

NCBI: 27241
MIM: 607968
HGNC: 30000
Ensembl: ENSG00000122507

Variants:

dbSNP: 27241
ClinVar: 27241
TCGA: ENSG00000122507
COSMIC: BBS9

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000122507ENST00000242067Q3SYG4
ENSG00000122507ENST00000242067A0A090N8P4
ENSG00000122507ENST00000350941Q3SYG4
ENSG00000122507ENST00000350941A0A090N7W2
ENSG00000122507ENST00000355070Q3SYG4
ENSG00000122507ENST00000396127Q3SYG4
ENSG00000122507ENST00000425508Q3SYG4
ENSG00000122507ENST00000432983C9JJ08
ENSG00000122507ENST00000433714F8WCG5
ENSG00000122507ENST00000434373H7BZ69

Expression (GTEx)

0
5
10
15
20

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
346928302021A Novel BBS9 Mutation Identified via Whole-Exome Sequencing in a Chinese Family with Bardet-Biedl Syndrome.2
346928302021A Novel BBS9 Mutation Identified via Whole-Exome Sequencing in a Chinese Family with Bardet-Biedl Syndrome.2
331380632020Next-Generation Sequencing in the Diagnosis of Patients with Bardet-Biedl Syndrome-New Variants and Relationship with Hyperglycemia and Insulin Resistance.9
331380632020Next-Generation Sequencing in the Diagnosis of Patients with Bardet-Biedl Syndrome-New Variants and Relationship with Hyperglycemia and Insulin Resistance.9
312945302019Exome sequence analysis in consanguineous Pakistani families inheriting Bardet-Biedle syndrome determined founder effect of mutation c.299delC (p.Ser100Leufs*24) in BBS9 gene.5
315306392019Molecular architecture of the Bardet-Biedl syndrome protein 2-7-9 subcomplex.5
312945302019Exome sequence analysis in consanguineous Pakistani families inheriting Bardet-Biedle syndrome determined founder effect of mutation c.299delC (p.Ser100Leufs*24) in BBS9 gene.5
315306392019Molecular architecture of the Bardet-Biedl syndrome protein 2-7-9 subcomplex.5
296741262018BBS9 gene in nonsyndromic craniosynostosis: Role of the primary cilium in the aberrant ossification of the suture osteogenic niche.7
296741262018BBS9 gene in nonsyndromic craniosynostosis: Role of the primary cilium in the aberrant ossification of the suture osteogenic niche.7
264809202016Genome-wide association studies suggest sex-specific loci associated with abdominal and visceral fat.48
268460962016Homozygosity mapping identified a novel protein truncating mutation (p.Ser100Leufs*24) of the BBS9 gene in a consanguineous Pakistani family with Bardet Biedl syndrome.9
264809202016Genome-wide association studies suggest sex-specific loci associated with abdominal and visceral fat.48
268460962016Homozygosity mapping identified a novel protein truncating mutation (p.Ser100Leufs*24) of the BBS9 gene in a consanguineous Pakistani family with Bardet Biedl syndrome.9
244006382015Mutation spectrum in BBS genes guided by homozygosity mapping in an Indian cohort.17

Citation

Dessen P

BBS9 (Bardet-Biedl syndrome 9)

Atlas Genet Cytogenet Oncol Haematol. 2007-02-01

Online version: http://atlasgeneticsoncology.org/gene/43610/bbs9-(bardet-biedl-syndrome-9)