AAI12892.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
71,472 Da
NCBI Official Full Name
SNRNP200 protein, partial
NCBI Official Synonym Full Names
small nuclear ribonucleoprotein U5 subunit 200
NCBI Official Synonym Symbols
BRR2; RP33; HELIC2; ASCC3L1; U5-200KD [Similar Products]
NCBI Protein Information
U5 small nuclear ribonucleoprotein 200 kDa helicase
UniProt Protein Name
U5 small nuclear ribonucleoprotein 200 kDa helicase
UniProt Synonym Protein Names
Activating signal cointegrator 1 complex subunit 3-like 1; BRR2 homolog; U5 snRNP-specific 200 kDa protein; U5-200KD
UniProt Synonym Gene Names
UniProt Entry Name
U520_HUMAN
NCBI Summary for SNRNP200
Pre-mRNA splicing is catalyzed by the spliceosome, a complex of specialized RNA and protein subunits that removes introns from a transcribed pre-mRNA segment. The spliceosome consists of small nuclear RNA proteins (snRNPs) U1, U2, U4, U5 and U6, together with approximately 80 conserved proteins. U5 snRNP contains nine specific proteins. This gene encodes one of the U5 snRNP-specific proteins. This protein belongs to the DEXH-box family of putative RNA helicases. It is a core component of U4/U6-U5 snRNPs and appears to catalyze an ATP-dependent unwinding of U4/U6 RNA duplices. Mutations in this gene cause autosomal-dominant retinitis pigmentosa. Alternatively spliced transcript variants encoding different isoforms have been found, but the full-length nature of these variants has not been determined. [provided by RefSeq, Mar 2010]
UniProt Comments for SNRNP200
U5-200kD: Putative RNA helicase involved in the second step of RNA splicing. May promote one or more conformational changes in the dynamic network of RNA-RNA interactions in the spliceosome. Appears to catalyze an ATP-dependent unwinding of U4/U6 RNA duplices. Defects in SNRNP200 are the cause of retinitis pigmentosa type 33 (RP33). It is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. Belongs to the helicase family. SKI2 subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: RNA splicing; Spliceosome; Helicase; RNA-binding; RNA processing; EC 3.6.4.13
Chromosomal Location of Human Ortholog: 2q11.2
Cellular Component: membrane; nucleoplasm; nucleus; snRNP U5; spliceosome
Molecular Function: ATP binding; ATP-dependent helicase activity; ATP-dependent RNA helicase activity; identical protein binding; protein binding
Biological Process: cis assembly of pre-catalytic spliceosome; nuclear mRNA splicing, via spliceosome; osteoblast differentiation
Disease: Retinitis Pigmentosa 33
Research Articles on SNRNP200
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Pathways associated with anti-SNRNP200 antibody
Diseases associated with anti-SNRNP200 antibody
Organs/Tissues associated with anti-SNRNP200 antibody
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