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troponin C, slow skeletal & cardiac muscles; TN-C (TNNC1 TNNC)
Function:
- troponin is the central regulatory protein of striated muscle contraction
- it consists of three components:
a) troponin-I, the inhibitor of actomyosin ATPase
b) troponin-T contains the binding site for tropomyosin
c) troponin-C
- binding of Ca+2 to troponin-C abolishes the inhibitory action of troponin on actin filaments
Structure:
- belongs to the troponin C family
- contains 4 EF-hand domains
Pathology:
- defects in TNNC1 are the cause of dilated cardiomyopathy type 1Z
Properties
GENERAL: troponin-C
SIZE: entity length = 161 aa
MW = 18 kD
FORM: troponin C, slow skeletal & cardiac muscles
MOTIF: acetylation site
SITE: N-TERMINUS
EFFECTOR-BOUND: acetyl
EF hand
SITE: 16-51
Ca+2-binding site (2)
SITE: N-TERMINAL
EF hand
SITE: 52-87
MOTIF: Ca+2-binding site
SITE: 65-76
EF hand
SITE: 92-127
MOTIF: Ca+2-binding site
SITE: 105-116
Ca+2-binding site (2)
SITE: C-TERMINAL
EFFECTOR-BOUND: Ca+2
EF hand
SITE: 128-161
MOTIF: Ca+2-binding site
SITE: 141-152
COMPONENT-OF: troponin
Database Correlations
OMIM correlations
MORBIDMAP 191040
UniProt P63316
Pfam PF00036
Entrez Gene 7134
Kegg hsa:7134
References
UniProt :accession P63316