Protein Domain : LAP1C-like, C-terminal domain superfamily IPR038599

Type  Homologous_superfamily
Description  This entry represents Torsin-1A-interacting proteins 1 and 2 (TOIP 1/2) also known as LAP1 proteins (Lamina-associated polypeptide 1), which are type 2 integral membrane proteins with a single membrane-spanning region of the inner nuclear membrane [ , , ]. These proteins interact with and activate Torsin A, an AAA+ ATPase localized to the endoplasmic reticulum (ER), through a perinuclear domain and forms a heterohexameric (LAP1-Torsin)3 ring that targets Torsin to the nuclear envelope. LAP1 has an atypical AAA+ fold and provides an arginine finger to the Torsin A active site to promote its ATPase activity [, ]. A single mutation in Torsin A causes early onset primary dystonia, a painful and severely disabling neuromuscular disease [, ].This is the conserved C-terminal domain of LAP1, the AAA Activator domain [, ].
Short Name  LAP1C-like_C_sf

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