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Gene | SMARCB1 |
Variant | T72Nfs*4 |
Impact List | frameshift |
Protein Effect | loss of function - predicted |
Gene Variant Descriptions | SMARCB1 T72Nfs*4 indicates a shift in the reading frame starting at amino acid 72 and terminating 4 residues downstream causing a premature truncation of the 385 amino acid Smarcb1 protein (UniProt.org). Due to the loss of the MYC-binding and tandem repeat regions (UniProt.org), T72Nfs*4 is predicted to lead to a loss of Smarcb1 protein function. |
Associated Drug Resistance | |
Category Variants Paths |
SMARCB1 mutant SMARCB1 inact mut SMARCB1 T72Nfs*4 |
Transcript | NM_003073.5 |
gDNA | chr22:g.23791876dupA |
cDNA | c.214dupA |
Protein | p.T72Nfs*4 |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_003073 | chr22:g.23791871_23791872insG | c.209_210insG | p.T72Nfs*4 | RefSeq | GRCh38/hg38 |
XM_011530345 | chr22:g.23791871_23791872insG | c.209_210insG | p.T72Nfs*4 | RefSeq | GRCh38/hg38 |
XM_011530345.2 | chr22:g.23791876dupA | c.214dupA | p.T72Nfs*4 | RefSeq | GRCh38/hg38 |
NM_001362877.2 | chr22:g.23791876dupA | c.214dupA | p.T72Nfs*4 | RefSeq | GRCh38/hg38 |
NM_003073.4 | chr22:g.23791876dupA | c.214dupA | p.T72Nfs*4 | RefSeq | GRCh38/hg38 |
NM_003073.5 | chr22:g.23791876dupA | c.214dupA | p.T72Nfs*4 | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
SMARCB1 inact mut | rhabdoid cancer | not applicable | N/A | Guideline | Diagnostic | SMARCB1 inactivating mutations aid the diagnosis of extrarenal rhabdoid tumor (NCCN.org). | detail... |
SMARCB1 inact mut | transitional cell carcinoma | not predictive | unspecified immune checkpoint inhibitor | Clinical Study - Cohort | Actionable | In a retrospective analysis, somatic loss-of-function mutations in the genes of the mSWI/SNF complex, including SMARCB1, did not correlate with improved survival in 2 separate cohorts of patients with transitional cell carcinoma treated with systemic immune checkpoint inhibitors, with adjusted HRs for overall survival of 1.44 (p=0.34, n=56) and 0.82 (p=0.559, n=93), respectively (PMID: 32321774). | 32321774 |
SMARCB1 inact mut | colorectal adenocarcinoma | unknown | unspecified immune checkpoint inhibitor | Clinical Study - Cohort | Actionable | In a retrospective analysis, somatic loss-of-function mutations in the genes of the mSWI/SNF complex, including ARID1A, correlated with improved survival in one cohort of patients with colorectal adenocarcinoma treated with systemic immune checkpoint inhibitors but not the other, with adjusted HRs for overall survival of 0.30 (p=0.03, n=35) and 0.56 (p=0.244, n=63), respectively (PMID: 32321774). | 32321774 |
SMARCB1 inact mut | head and neck squamous cell carcinoma | not predictive | unspecified immune checkpoint inhibitor | Clinical Study - Cohort | Actionable | In a retrospective analysis, somatic loss-of-function mutations in the genes of the mSWI/SNF complex, including SMARCB1, did not correlate with improved survival in 2 separate cohorts of patients with head and neck squamous cell carcinoma treated with systemic immune checkpoint inhibitors, with adjusted HRs for overall survival of 0.74 (p=0.631, n=31) and 0.76 (p=0.622, n=68), respectively (PMID: 32321774). | 32321774 |
SMARCB1 inact mut | epithelioid sarcoma | not applicable | N/A | Guideline | Diagnostic | SMARCB1 inactivating mutations aid the diagnosis of epithelioid sarcoma (NCCN.org). | detail... |
SMARCB1 inact mut | melanoma | not predictive | unspecified immune checkpoint inhibitor | Clinical Study - Cohort | Actionable | In a retrospective analysis, somatic loss-of-function mutations in the genes of the mSWI/SNF complex, including SMARCB1, did not correlate with improved survival in 2 separate cohorts of patients with melanoma treated with systemic immune checkpoint inhibitors, with adjusted HRs for overall survival of 1.70 (p=0.192, n=86) and 1.02 (p=0.939, n=192), respectively (PMID: 32321774). | 32321774 |
SMARCB1 inact mut | gastroesophageal adenocarcinoma | not predictive | unspecified immune checkpoint inhibitor | Clinical Study - Cohort | Actionable | In a retrospective analysis, somatic loss-of-function mutations in the genes of the mSWI/SNF complex, including SMARCB1, did not correlate with improved survival in 2 separate cohorts of patients with gastroesophageal adenocarcinoma treated with systemic immune checkpoint inhibitors, with adjusted HRs for overall survival of 0.70 (p=0.403, n=66) and 0.46 (p=0.071, n=59), respectively (PMID: 32321774). | 32321774 |