FLNA Chromosome X

Filamin A
695 variants 695 Health Risk

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What This Gene Does
The protein encoded by this gene is an actin-binding protein that crosslinks actin filaments and links actin filaments to membrane glycoproteins. The encoded protein is involved in remodeling the cytoskeleton to effect changes in cell shape and migration. This protein interacts with integrins, transmembrane receptor complexes, and second messengers. Defects in this gene are a cause of several syndromes, including periventricular nodular heterotopias (PVNH1, PVNH4), otopalatodigital syndromes (OPD1, OPD2), frontometaphyseal dysplasia (FMD), Melnick-Needles syndrome (MNS), and X-linked congenital idiopathic intestinal pseudoobstruction (CIIPX). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2009]
Gene Info
Gene Group
Filamin family
Locus Type
gene with protein product
Location
Xq28
Ensembl
ENSG00000196924
Associated Conditions (77)
Melnick-Needles syndrome
Oto-palato-digital syndrome
type II
Heterotopia
periventricular
X-linked dominant
Frontometaphyseal dysplasia
Familial thoracic aortic aneurysm and aortic dissection
Nonpapillary renal cell carcinoma
Thyroid cancer
nonmedullary
1
Thoracic aortic aneurysm or dissection
FLNA-related disorder
Cardiac valvular dysplasia
X-linked
Neurodevelopmental delay
type I
History of neurodevelopmental disorder
9 conditions
+57 more conditions
Key Variants
RS1004772663
Conflicting classifications of pathogenicity
Melnick-Needles syndrome, Oto-palato-digital syndrome, type II
Health Risk
RS10458342
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Heterotopia, periventricular
Health Risk
RS1057518479
Conflicting classifications of pathogenicity
Frontometaphyseal dysplasia, Melnick-Needles syndrome, Heterotopia
Health Risk
RS1057520770
Conflicting classifications of pathogenicity
Frontometaphyseal dysplasia, Melnick-Needles syndrome, Heterotopia
Health Risk
RS1057524317
Conflicting classifications of pathogenicity
Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
Health Risk
RS1057524434
Conflicting classifications of pathogenicity
Frontometaphyseal dysplasia, Heterotopia, periventricular
Health Risk
RS1057524473
Conflicting classifications of pathogenicity
Melnick-Needles syndrome, Frontometaphyseal dysplasia, Heterotopia
Health Risk
RS1064796297
Conflicting classifications of pathogenicity
Thoracic aortic aneurysm or dissection, Thoracic aortic aneurysm or dissection
Health Risk
RS1085307783
Conflicting classifications of pathogenicity
Oto-palato-digital syndrome, type II, Frontometaphyseal dysplasia
Health Risk
RS111516546
Conflicting classifications of pathogenicity
Heterotopia, periventricular, X-linked dominant
Health Risk
RS1131691935
Conflicting classifications of pathogenicity
Frontometaphyseal dysplasia, Melnick-Needles syndrome, Heterotopia
Health Risk
RS1172505050
Conflicting classifications of pathogenicity
Heterotopia, periventricular, X-linked dominant
Health Risk
All Variants (695)
RSID Category Clinical Significance Conditions
RS1557178694 Health Risk Likely pathogenic
RS1557179684 Health Risk Likely pathogenic Familial thoracic aortic aneurysm and aortic dissection, Familial thoracic aortic aneurysm and aortic dissection
RS1557180206 Health Risk Likely pathogenic
RS1569551449 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS1603359091 Health Risk Likely pathogenic Prune belly syndrome, Prune belly syndrome
RS1603359205 Health Risk Likely pathogenic Prune belly syndrome, Prune belly syndrome
RS1603359417 Health Risk Likely pathogenic
RS1603360906 Health Risk Likely pathogenic Melnick-Needles syndrome, Frontometaphyseal dysplasia, Heterotopia
RS2067597172 Health Risk Likely pathogenic Melnick-Needles syndrome, Heterotopia, periventricular
RS2067713488 Health Risk Likely pathogenic Melnick-Needles syndrome, Heterotopia, periventricular
RS2067762985 Health Risk Likely pathogenic Melnick-Needles syndrome, Heterotopia, periventricular
RS2067774568 Health Risk Likely pathogenic Cardiac valvular dysplasia, X-linked, Cardiac valvular dysplasia
RS2148102202 Health Risk Likely pathogenic Oto-palato-digital syndrome, type II, Oto-palato-digital syndrome
RS2148102980 Health Risk Likely pathogenic Frontometaphyseal dysplasia, Oto-palato-digital syndrome, type II
RS2148104272 Health Risk Likely pathogenic Attenuated frontometaphyseal dysplasia, Attenuated frontometaphyseal dysplasia
RS2148104376 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS2148105341 Health Risk Likely pathogenic Macrothrombocytopenia, Macrothrombocytopenia
RS2148107380 Health Risk Likely pathogenic Melnick-Needles syndrome, Melnick-Needles syndrome
RS2148107588 Health Risk Likely pathogenic
RS2148114607 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS2148115834 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS2148119316 Health Risk Likely pathogenic Melnick-Needles syndrome, Frontometaphyseal dysplasia, Heterotopia
RS2148119399 Health Risk Likely pathogenic See cases, See cases
RS2148121838 Health Risk Likely pathogenic Periventricular nodular heterotopia, Periventricular nodular heterotopia
RS2148121877 Health Risk Likely pathogenic
RS2148121932 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS2522712579 Health Risk Likely pathogenic Frontometaphyseal dysplasia 1, Frontometaphyseal dysplasia 1
RS2522714778 Health Risk Likely pathogenic FLNA-related disorder, Cardiac valvular dysplasia, X-linked
RS2522719482 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
RS2522719685 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
RS2522726645 Health Risk Likely pathogenic Oto-palato-digital syndrome, type II, Heterotopia
RS2522733678 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
RS2522737559 Health Risk Likely pathogenic
RS2522740256 Health Risk Likely pathogenic Melnick-Needles syndrome, Oto-palato-digital syndrome, type II
RS2522740258 Health Risk Likely pathogenic Oto-palato-digital syndrome, type II, Melnick-Needles syndrome
RS2522741003 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS2522741040 Health Risk Likely pathogenic
RS2522741180 Health Risk Likely pathogenic FG syndrome 2, FG syndrome 2
RS2522747986 Health Risk Likely pathogenic
RS2522749173 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS2522753919 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
RS2522760708 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
RS2522760720 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
RS2522761063 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS2522763814 Health Risk Likely pathogenic Frontometaphyseal dysplasia, Oto-palato-digital syndrome, type II
RS2522765595 Health Risk Likely pathogenic Heterotopia, periventricular, X-linked dominant
RS2522771398 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
RS2522771411 Health Risk Likely pathogenic Oto-palato-digital syndrome, type II, Frontometaphyseal dysplasia
RS2522771783 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
RS2522771899 Health Risk Likely pathogenic FLNA-related disorder, FLNA-related disorder
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