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Protein basic information

LiverAtlas Protein ID

HuLPr46494

Uniprot ID

SYRM_HUMAN

Uniprot Acc

Q5T160;B2RDT7;Q96FU5;Q9H8K8;

Protein name

Probable arginyl-tRNA synthetase, mitochondrial

Comment

CATALYTIC ACTIVITY:ATP + L-arginine + tRNA(Arg) = AMP + diphosphate + L-arginyl-tRNA(Arg).||INTERACTION:Q9Y5J5:PHLDA3; NbExp=1; IntAct=EBI-1050546, EBI-1055859;||SUBCELLULAR LOCATION:Mitochondrion matrix (By similarity).||DISEASE:Defects in RARS2 are the cause of pontocerebellar hypoplasia type 6 (PCH6) [MIM:611523]; also known as fatal infantile encephalopathy with mitochondrial respiratory chain defects. Pontocerebellar hypoplasia (PCH) is a heterogeneous group of disorders characterized by an abnormally small cerebellum and brainstem.||SIMILARITY:Belongs to the class-I aminoacyl-tRNA synthetase family.

Subcellular localization

Mitochondrion matrix(By similarity).

Gene name

arginyl-tRNA synthetase 2, mitochondrial

Protein sequence

MACGFRRAIACQLSRVLNLPPENLITSISAVPISQKEEVA DFQLSVDSLLEKDNDHSRPDIQVQAKRLAEKLRCDTVVS EISTGQRTVNFKINRELLTKTVLQQVIEDGSKYGLKSEL FSGLPQKKIVVEFSSPNVAKKFHVGHLRSTIIGNFIANL KEALGHQVIRINYLGDWGMQFGLLGTGFQLFGYEEKLQS NPLQHLFEVYVQVNKEAADDKSVAKAAQEFFQRLELGDV QALSLWQKFRDLSIEEYIRVYKRLGVYFDEYSGESFYRE KSQEVLKLLESKGLLLKTIKGTAVVDLSGNGDPSSICTV MRSDGTSLYATRDLAAAIDRMDKYNFDTMIYVTDKGQKK HFQQVFQMLKIMGYDWAERCQHVPFGVVQGMKTRRGDVT FLEDVLNEIQLRMLQNMASIKTTKELKNPQETAERVGLA ALIIQDFKGLLLSDYKFSWDRVFQSRGDTGVFLQYTHAR LHSLEETFGCGYLNDFNTACLQEPQSVSILQHLLRFDEV LYKSSQDFQPRHIVSYLLTLSHLAAVAHKTLQIKDSPPE VAGARLHLFKAVRSVLANGMKLLGITPVCRM

Database cross reference

RefSeq Protein accession:NP_064716
RefSeq Protein gi:197100773

Liver relevance

HLPP validation

Yes/No

Yes

Project name

Chinese Liver;Human Liver Organelles;

Ontology annotation

GO-C

GO:0005759;C:mitochondrial matrix;EXP:Reactome.

GO-F

GO:0004814;F:arginine-tRNA ligase activity;EXP:Reactome. GO:0005524;F:ATP binding;IEA:UniProtKB-KW. GO:0005515;F:protein binding;IPI:IntAct.

GO-P

GO:0006420;P:arginyl-tRNA aminoacylation;IEA:InterPro.

Pathway

Pathway name

Arginine_and_Proline_Metabolism

Pathway name

Prolidase_Deficiency(PD)