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H/ACA ribonucleoprotein complex subunit 1

 
Known also as: Nucleolar protein family A member 1, snoRNP protein GAR1

Known abbreviations: GAR1, NOLA1

Function

Required for ribosome biogenesis and telomere maintenance. Part of the H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine ("psi") residues, which may serve to stabilize the conformation of rRNAs. May also be required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme.

Subunit structure

Part of the H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which contains NHP2/NOLA2, GAR1/NOLA1, NOP10/NOLA3, and DKC1/NOLA4, which is presumed to be the catalytic subunit. The complex contains a stable core formed by binding of one or two NOP10-DKC1 heterodimers to NHP2; GAR1 subsequently binds to this core via DKC1. The complex binds a box H/ACA small nucleolar RNA (snoRNA), which may target the specific site of modification within the RNA substrate. The complex also interacts with TERC, which contains a 3'-terminal domain related to the box H/ACA snoRNAs. Specific interactions with snoRNAs or TERC are mediated by GAR1 and NHP2. Associates with NOLC1/NOPP140. H/ACA snoRNPs interact with the SMN complex, consisting of SMN1 or SMN2, GEMIN2/SIP1, DDX20/GEMIN3, and GEMIN4. This is mediated by interaction between GAR1 and SMN1 or SMN2. The SMN complex may be required for correct assembly of the H/ACA snoRNP complex. Component of the telomerase holoenzyme complex at least composed of TERT, DKC1, WRAP53/TCAB1, NOP10, NHP2, GAR1, TEP1, EST1A, POT1 and a telomerase RNA template component (TERC).

Subcellular location

Nucleus › nucleolus. Nucleus › Cajal body. Note: Also localized to Cajal bodies (coiled bodies).

Domain

Interaction with SMN1 requires at least one of the RGG-box regions.

Sequence similarities

Belongs to the GAR1 family.




Activities in which H/ACA ribonucleoprotein complex subunit 1 is involved: Pathways in which H/ACA ribonucleoprotein complex subunit 1 is involved:

NCBI GI number(s): -
Species: Homo sapiens

Links to other databases:

Database ID Link
Uniprot Q9NY12 Q9NY12
BRENDA - -
KEGG hsa:54433 hsa:54433
PFAM: PF04410
PF04410
InterPro: IPR007504
IPR021154
IPR009000
IPR007504
IPR021154
IPR009000
CATH: - -
SCOP: - -


Protein sequence:
MSFRGGGRGGFNRGGGGGGFNRGGSSNHFRGGGGGGGGGNFRGGGRGGFG
RGGGRGGFNKGQDQGPPERVVLLGEFLHPCEDDIVCKCTTDENKVPYFNA
PVYLENKEQIGKVDEIFGQLRDFYFSVKLSENMKASSFKKLQKFYIDPYK
LLPLQRFLPRPPGEKGPPRGGGRGGRGGGRGGGGRGGGRGGGFRGGRGGG
GGGFRGGRGGGFRGRGH

H/ACA ribonucleoprotein complex subunit 1 (Homo sapiens) is product of expression of GAR1 ribonucleoprotein homolog (yeast) gene.



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Last modification of this entry: May 1, 2013.

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