Domain

Alpha Spectrin, SH3 domain (IPR035825)

Short name: Alpha_Spectrin_SH3

Overlapping homologous superfamilies

Domain relationships

  • SH3 domain (IPR001452)
    • Alpha Spectrin, SH3 domain (IPR035825)

Description

Spectrin is a major structural component of the red blood cell membrane skeleton and is important in erythropoiesis and membrane biogenesis. It is a flexible, rope-like molecule composed of two subunits, alpha and beta, which consist of many spectrin-type repeats. Alpha and beta spectrin associate to form heterodimers and tetramers; spectrin tetramer formation is critical for red cell shape and deformability [PMID: 11427698, PMID: 12820899]. Defects in alpha spectrin cause West syndrome with severe cerebral hypomyelination, spastic quadriplegia, and developmental delay [PMID: 20493457]. Alpha spectrin contains a middle SH3 domain and a C-terminal EF-hand binding motif in addition to multiple spectrin repeats.

This entry includes the SH3 domain of spectrin.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
CDD