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MT001138

Record overview

MITO IDMT001138
Gene ID55157
SpeciesHomo sapiens (Human)
Gene Nameaspartyl-tRNA synthetase 2, mitochondrial
Gene SymbolDARS2
SynonymsLBSL; ASPRS; MT-ASPRS;
Alternate namesaspartate--tRNA ligase, mitochondrial; aspartate tRNA ligase 2, mitochondrial; aspartyl-tRNA synthetase, mitochondrial;
Chromosome1
Map Location1q25.1
EC Number6.1.1.12
SummaryThe protein encoded by this gene belongs to the class-II aminoacyl-tRNA synthetase family. It is a mitochondrial enzyme that specifically aminoacylates aspartyl-tRNA. Mutations in this gene are associated with leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL). [provided by RefSeq, Nov 2009]
OrthologsView orthologs and multiple alignments for DARS2

Proteins

aspartate--tRNA ligase, mitochondrial
Refseq ID:NP_060592
Protein GI:40789249
UniProt ID:Q6PI48
mRNA ID:NM_018122
Length:645
RefSeq Status:
MYFPSWLSQLYRGLSRPIRRTTQPIWGSLYRSLLQSSQRRIPEFSSFVVRTNTCGELRSSHLGQEVTLCGWIQYRRQNTFLVLRDFDGLVQVIIPQDESA
ASVKKILCEAPVESVVQVSGTVISRPAGQENPKMPTGEIEIKVKTAELLNACKKLPFEIKNFVKKTEALRLQYRYLDLRSFQMQYNLRLRSQMVMKMREY
LCNLHGFVDIETPTLFKRTPGGAKEFLVPSREPGKFYSLPQSPQQFKQLLMVGGLDRYFQVARCYRDEGSRPDRQPEFTQIDIEMSFVDQTGIQSLIEGL
LQYSWPNDKDPVVVPFPTMTFAEVLATYGTDKPDTRFGMKIIDISDVFRNTEIGFLQDALSKPHGTVKAICIPEGAKYLKRKDIESIRNFAADHFNQEIL
PVFLNANRNWNSPVANFIMESQRLELIRLMETQEEDVVLLTAGEHNKACSLLGKLRLECADLLETRGVVLRDPTLFSFLWVVDFPLFLPKEENPRELESA
HHPFTAPHPSDIHLLYTEPKKARSQHYDLVLNGNEIGGGSIRIHNAELQRYILATLLKEDVKMLSHLLQALDYGAPPHGGIALGLDRLICLVTGSPSIRD
VIAFPKSFRGHDLMSNTPDSVPPEELKPYHIRVSKPTDSKAERAH