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Gene | TSC2 |
Variant | V504D |
Impact List | missense |
Protein Effect | loss of function |
Gene Variant Descriptions | TSC2 V504D does not lie within any known functional domains of the Tsc2 protein (UniProt.org). V504D fails to inhibit Mtorc1 activity as indicated by reduced inhibition of S6k phosphorylation and leads to decreased interaction with Tsc1 in cultured cells (PMID: 31799751). |
Associated Drug Resistance | |
Category Variants Paths |
TSC2 mutant TSC2 inact mut TSC2 V504D |
Transcript | NM_000548.5 |
gDNA | chr16:g.2064339T>A |
cDNA | c.1511T>A |
Protein | p.V504D |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_000548.4 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_017023615.1 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_011522637.2 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001077183.2 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_017023616.1 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_000548.5 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_011522636.3 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_011522639.2 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_011522636.2 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001370404.1 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001114382.3 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001363528.2 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_024450413.1 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_021055.3 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001406665.1 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001406664.1 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001406663.1 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_005255529.4 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_011522640.2 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_005255531.4 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_011522639.3 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
XM_011522637.3 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001370405.1 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001114382.2 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
NM_001077183.3 | chr16:g.2064339T>A | c.1511T>A | p.V504D | RefSeq | GRCh38/hg38 |
Molecular Profile | Protein Effect | Treatment Approaches |
---|---|---|
TSC2 V504D | loss of function | mTOR Inhibitor mTORC1 Inhibitor |