Protein Snrnp200

URN	urn:agi-llid:23020
Total Entities	0
Connectivity	15
Name	Snrnp200
Description	small nuclear ribonucleoprotein 200kDa (U5)
Notes	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]

GO Molecular Function	ATP-dependent helicase activity
	ATP-dependent RNA helicase activity
	hydrolase activity
	nucleoside-triphosphatase activity
	helicase activity
	nucleotide binding
	ATP binding
	nucleic acid binding

GO Biological Process	ATP catabolic process
	RNA splicing
	mRNA processing
	mRNA splicing, via spliceosome
	cis assembly of pre-catalytic spliceosome
	gene expression

Pathway	spliceosomal pathway
	spliceosome pathway

Group	Glucose metabolism
	mRNA processing
	ATP catabolic process
	ATP-dependent helicase activity
	ATP-dependent RNA helicase activity
	hydrolase activity
	nucleoside-triphosphatase activity
	helicase activity
	RNA splicing
	mRNA processing
	mRNA splicing, via spliceosome
	cis assembly of pre-catalytic spliceosome
	gene expression
	nucleotide binding
	ATP binding
	nucleic acid binding
	spliceosomal complex
	nucleoplasm
	nucleolus
	catalytic step 2 spliceosome
	U5 snRNP
	ribonucleoprotein complex
	nucleus

Cell Localization	Cytoplasm
	Nucleus

Alias	BRR2
	RP33
	HELIC2
	ASCC3L1
	U5-200KD
	U5 small nuclear ribonucleoprotein 200 kDa helicase
	BRR2 homolog
	U5 snRNP-specific 200 kDa protein
	activating signal cointegrator 1 complex subunit 3-like 1
	BC011390
	U5-200-KD
	A330064G03Rik
	RP23-206D14.2
	small nuclear ribonucleoprotein 200kDa (U5)
	U5 snRNP-specific protein, 200 kDa (DEXH RNA helicase family)
	KIAA0788
	RP33 locus
	U5 snRNP specific protein, 200 KD
	u5 snrnp-specific protein, 200 kda
	MGC116386
	retinitis pigmentosa 33 locus
	RP33 gene
	small nuclear ribonucleoprotein 200 (U5)
	locus RP33
	Snrnp200
	activating signal cointegrator 1 complex subunit 3-like I
	cDNA sequence BC011390
	OTTMUSP00000017113
	retinitis pigmentosa 33 (autosomal dominant)
	FLJ11521

Mouse chromosome position	2 61.84 cM

OMIM ID	601664
	610359

Rat chromosome position	3q36

Hugo ID	30859

Human chromosome position	2q11.2

Swiss-Prot ID	U520_HUMAN
	U520_MOUSE

EC Number	3.6.4.13

Homologene ID	5859

MGI ID	2444401

RGD ID	1561120

MedScan ID	23020