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TNNT3

Function

Troponin T is the tropomyosin-binding subunit of troponin, the thin filament regulatory complex which confers calcium-sensitivity to striated muscle actomyosin ATPase activity.

Involvement in disease

Arthrogryposis, distal, 2B2

DA2B2

A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. Distal arthrogryposis type 2 is characterized by contractures of the hands and feet, and a distinctive face characterized by prominent nasolabial folds, small mouth and downslanting palpebral fissures. DA2B2 inheritance is autosomal dominant.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the troponin T family.

Tissue Specificity

In fetal and adult fast skeletal muscles, with a higher level expression in fetal than in adult muscle.

Alternative names

TnTf, Beta-TnTF, Fast skeletal muscle troponin T, fTnT, TNNT3

swissprot:P45378 omim:600692 entrezGene:7140