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spectrin alpha chain, erythrocyte; erythroid alpha-spectrin (SPTA1, SPTA)
Function:
- spectrin alpha subunit
Structure:
- belongs to the spectrin family
- contains 3 EF-hand domains
- contains 1 SH3 domain
- contains 22 spectrin repeats
Compartment:
- cytoplasm, cytoskeleton. cytoplasm, cell cortex
Pathology:
- defects in SPTA1 are the cause of:
a) elliptocytosis type 2
b) hereditary pyropoikilocytosis
c) hereditary spherocytosis type 3
General
protein subunit
Properties
SIZE: entity length = 2418 aa
MW = 280 kD
COMPARTMENT: cytoplasm
MOTIF: spectrin repeat {19-51}
spectrin repeat {53-156}
spectrin repeat {158-262}
spectrin repeat {264-368}
spectrin repeat {370-474}
spectrin repeat {476-580}
spectrin repeat {582-685}
spectrin repeat {687-791}
spectrin repeat {793-897}
spectrin repeat {899-975}
src homology 3 [SH3] domain
SITE: 977-1036
spectrin repeat {1057-1080}
spectrin repeat {1082-1181}
spectrin repeat {1183-1287}
spectrin repeat {1289-1393}
spectrin repeat {1395-1498}
spectrin repeat {1500-1604}
spectrin repeat {1606-1710}
spectrin repeat {1712-1816}
spectrin repeat {1818-1925}
spectrin repeat {1927-2032}
spectrin repeat {2042-2146}
spectrin repeat {2156-2257}
EF hand
SITE: 2270-2305
MOTIF: Ca+2-binding site
SITE: 2283-2294
EF hand
SITE: 2313-2348
MOTIF: Ca+2-binding site
SITE: 2326-2337
EF hand
SITE: 2351-2385
Database Correlations
OMIM correlations
MORBIDMAP 182860
UniProt P02549
PFAM correlations
Entrez Gene 6708
Kegg hsa:6708
References
UniProt :accession P02549
OMIM :accession 182860
Component-of
spectrin