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ubiquilin-1; protein linking IAP with cytoskeleton 1; PLIC-1; hPLIC-1 (UBQLN1, DA41, PLIC1)

Function: - links CD47 to the cytoskeleton - promotes the surface expression of GABA-A receptors - promotes accumulation of uncleaved PSEN1 & PSEN2 by stimulating their biosynthesis - has no effect on PSEN1 & PSEN2 degradation - phosphorylated - binds CD47, NBL1, GABRA1, GABRA2, GABRA3, GABRA6, GABRB1, GABRB2 & GABRB3 (putative). binds UBE3A, BTRC, P4HB, FRAP1, PSEN1 & PSEN2 - interacts with the proteasome 19S subunit Structure: - binds PSEN1 & PSEN2 via its UBA domain - contains 1 UBA domain - contains 1 ubiquitin-like domain Compartment: cytoplasm, nucleus Alternative splicing: named isoforms=2 Expression: - ubiquitous - highly expressed throughout the brain - detected in neurons - highly expressed in heart, placenta, pancreas, lung, liver, skeletal muscle & kidney Pathology: - detected in neuropathological lesions, including a) neurofibrillary tangles b) Lewy bodies Genetics: - gene on chromosome 9 Notes: - name derived from similarities with ubiquitin

General

nuclear protein ubiquilin

Properties

SIZE: entity length = 589 aa MW = 63 kD COMPARTMENT: cytoplasm cell nucleus MOTIF: acetylation site SITE: N-TERMINUS EFFECTOR-BOUND: acetyl Ubiquitin-like {37-111} UBA domain {546-586}

Database Correlations

OMIM 605046 UniProt Q9UMX0 PFAM correlations Entrez Gene 29979 Kegg hsa:29979

References

  1. Tanzi RE. In: Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 29-Oct 2, 2004
  2. UniProt :accession :Q9UMX0