TNNT1 Chromosome 19

Troponin T1, slow skeletal type
46 variants 46 Health Risk

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What This Gene Does
This gene encodes a protein that is a subunit of troponin, which is a regulatory complex located on the thin filament of the sarcomere. This complex regulates striated muscle contraction in response to fluctuations in intracellular calcium concentration. This complex is composed of three subunits: troponin C, which binds calcium, troponin T, which binds tropomyosin, and troponin I, which is an inhibitory subunit. This protein is the slow skeletal troponin T subunit. Mutations in this gene cause nemaline myopathy type 5, also known as Amish nemaline myopathy, a neuromuscular disorder characterized by muscle weakness and rod-shaped, or nemaline, inclusions in skeletal muscle fibers which affects infants, resulting in death due to respiratory insufficiency, usually in the second year. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
Gene Info
Gene Group
Troponin complex subunits
Locus Type
gene with protein product
Location
19q13.42
Ensembl
ENSG00000105048
Associated Conditions (9)
Nemaline myopathy 5
Inborn genetic diseases
TNNT1-related disorder
Ovarian serous cystadenocarcinoma
Nemaline myopathy 5C
autosomal dominant
Nemaline myopathy 5B
autosomal recessive
childhood-onset
Key Variants
All Variants (46)
RSID Category Clinical Significance Conditions
RS115663668 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS138664823 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS1461051435 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS1555859876 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Inborn genetic diseases, Nemaline myopathy 5
RS192248377 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS199652942 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Inborn genetic diseases, TNNT1-related disorder
RS201143291 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS201722303 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Inborn genetic diseases, Nemaline myopathy 5
RS2085462282 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS34313388 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS375682055 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Ovarian serous cystadenocarcinoma, Nemaline myopathy 5
RS375999352 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS376456377 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5, Nemaline myopathy 5
RS759885491 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS944152647 Health Risk Conflicting classifications of pathogenicity Nemaline myopathy 5, Nemaline myopathy 5
RS1156410888 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS1555855228 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2085575423 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2147253798 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2515403750 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5C, autosomal dominant
RS2515441946 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2515443940 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5B, autosomal recessive
RS2515470310 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2515474466 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS766934517 Health Risk Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS1166914763 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS1224321111 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS1250741190 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS1401047759 Health Risk Pathogenic
RS1555859304 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS1599896616 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2085299661 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2085439767 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2085561415 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2147244480 Health Risk Pathogenic
RS2515403962 Health Risk Pathogenic Nemaline myopathy 5B, autosomal recessive, childhood-onset
RS2515404859 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2515421573 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS2515444704 Health Risk Pathogenic Nemaline myopathy 5C, autosomal dominant, Nemaline myopathy 5C
RS727504177 Health Risk Pathogenic
RS759458391 Health Risk Pathogenic Nemaline myopathy 5B, autosomal recessive, childhood-onset
RS80358249 Health Risk Pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS149559898 Health Risk Pathogenic/Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS1599875856 Health Risk Pathogenic/Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
RS199701688 Health Risk Pathogenic/Likely pathogenic Nemaline myopathy 5B, autosomal recessive, childhood-onset
RS2085385176 Health Risk Pathogenic/Likely pathogenic Nemaline myopathy 5, Nemaline myopathy 5
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