DARS2 Antibody, HRP conjugated (PACO46663)
- SKU:
- PACO46663
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- HRP
Frequently bought together:
Description
抗体名: | DARS2 Antibody, HRP conjugated (PACO46663) |
抗体コード: | PACO46663 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Aspartate--tRNA ligase, mitochondrial protein (489-628AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | HRP |
バックグラウンド: | mitochondrial matrix, mitochondrion, nucleus, aspartate-tRNA ligase activity, aspartate-tRNA(Asn) ligase activity, ATP binding, protein homodimerization activity, tRNA binding, mitochondrial asparaginyl-tRNA aminoacylation, tRNA aminoacylation |
シノニム: | Aspartate--tRNA ligase, mitochondrial (EC 6.1.1.12) (Aspartyl-tRNA synthetase) (AspRS), DARS2 |
UniProt Protein Function: | DARS2: Defects in DARS2 are a cause of leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL). LBSL is an autosomal recessive disease and is defined on the basis of a highly characteristic constellation of abnormalities observed by magnetic resonance imaging and spectroscopy. Affected individuals develop slowly progressive cerebellar ataxia, spasticity, and dorsal column dysfunction, sometimes with a mild cognitive deficit or decline. Belongs to the class-II aminoacyl-tRNA synthetase family. |
UniProt Protein Details: | Protein type:Ligase; Translation; EC 6.1.1.12; Mitochondrial Chromosomal Location of Human Ortholog: 1q25.1 Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; nucleus Molecular Function:protein binding; protein homodimerization activity; aspartate-tRNA ligase activity; aspartate-tRNA(Asn) ligase activity; ATP binding; tRNA binding Biological Process: tRNA aminoacylation for protein translation; gene expression; tRNA aminoacylation Disease: Leukoencephalopathy With Brainstem And Spinal Cord Involvement And Lactate Elevation |
NCBI Summary: | The 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] |
UniProt Code: | Q6PI48 |
NCBI GenInfo Identifier: | 74758347 |
NCBI Gene ID: | 55157 |
NCBI Accession: | Q6PI48.1 |
UniProt Related Accession: | Q6PI48 |
Molecular Weight: | Observed: 68 kDaPredicted: 74 kDa |
NCBI Full Name: | Aspartate--tRNA ligase, mitochondrial |
NCBI Synonym Full Names: | aspartyl-tRNA synthetase 2, mitochondrial |
NCBI Official Symbol: | DARS2 |
NCBI Official Synonym Symbols: | LBSL; ASPRS; mtAspRS; MT-ASPRS |
NCBI Protein Information: | aspartate--tRNA ligase, mitochondrial |
UniProt Protein Name: | Aspartate--tRNA ligase, mitochondrial |
UniProt Synonym Protein Names: | Aspartyl-tRNA synthetase; AspRS |
UniProt Gene Name: | DARS2 |
UniProt Entry Name: | SYDM_HUMAN |