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NSFL1 cofactor p47 (NSFL1C)

Target: NSFL1C
Molecular classification: Other (UBX domain-containing adaptor protein)

Overview

NSFL1 cofactor p47, encoded by the NSFL1C gene, is a UBX domain-containing adaptor protein known for modulating the activity of the AAA ATPase valosin-containing protein (p97/VCP) by reducing its ATPase activity. It is essential for p97-mediated membrane fusion events, particularly in the fragmentation and reassembly of the Golgi apparatus during mitosis. NSFL1C also plays roles in regulating the localization of Aurora kinase A at the centrosome during cell division and spindle orientation, and may participate in transitional endoplasmic reticulum (tER) formation. Diseases associated with NSFL1C dysfunction include inclusion body myopathy with Paget disease of bone and frontotemporal dementia, and multisystem proteinopathy[1][2][3][5][6].

Other names: NSFL1 cofactorNSFL1CNSFL1 cofactor p47UBXN2CdJ776F14.1p47UBXD10UBX1UBX domain-containing protein 2Cp97 cofactor p47SHP1 homolog (S. cerevisiae)UBX domain protein 2CP47NSFL1 (p97) cofactor (p47)SHP1 homolog

Biological functions

Regulates vesicle/membrane fusionGolgi apparatus disassembly and reassembly during mitosisReduction of ATPase activity of VCP (also known as p97)Regulation of spindle orientationRegulation of Aurora kinase A (AURKA) localization

Disease associations

Inclusion body myopathy with Paget disease of bone and frontotemporal dementiaMultisystem proteinopathy

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