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Gene | VHL |
Variant | R64P |
Impact List | missense |
Protein Effect | loss of function - predicted |
Gene Variant Descriptions | VHL R64P does not lie within any known functional domains of the Vhl protein (UniProt.org). R64P demonstrates regulation of Hif2a and Glut1 similar to wild-type Vhl (PMID: 16452184, PMID: 16098468), however, results in disrupted fibronectin matrix assembly (PMID: 11331612, PMID: 16452184) and mitochondrial biogenesis (PMID: 35760869), increased Prkcz (aPKC) and Junb levels in cultured cells (PMID: 16098468), and increased tumor vascularization compared to controls in mouse models (PMID: 16452184), and therefore, is predicted to lead to a loss of Vhl protein function. |
Associated Drug Resistance | |
Category Variants Paths |
VHL mutant VHL inact mut VHL R64P |
Transcript | NM_000551.4 |
gDNA | chr3:g.10142038G>C |
cDNA | c.191G>C |
Protein | p.R64P |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_001354723.1 | chr3:g.10142038G>C | c.191G>C | p.R64P | RefSeq | GRCh38/hg38 |
NM_000551.4 | chr3:g.10142038G>C | c.191G>C | p.R64P | RefSeq | GRCh38/hg38 |
NM_000551.3 | chr3:g.10142038G>C | c.191G>C | p.R64P | RefSeq | GRCh38/hg38 |
NM_198156.2 | chr3:g.10142038G>C | c.191G>C | p.R64P | RefSeq | GRCh38/hg38 |
NM_198156 | chr3:g.10142038G>C | c.191G>C | p.R64P | RefSeq | GRCh38/hg38 |
NM_198156.3 | chr3:g.10142038G>C | c.191G>C | p.R64P | RefSeq | GRCh38/hg38 |
NM_000551 | chr3:g.10142038G>C | c.191G>C | p.R64P | RefSeq | GRCh38/hg38 |
NM_001354723.2 | chr3:g.10142038G>C | c.191G>C | p.R64P | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
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