Anti-DARS2 Antibody (CAB7813)
- SKU:
- CAB7813
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
抗体名: | Anti-DARS2 Antibody |
抗体コード: | CAB7813 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 406-645 of human DARS2 (NP_060592.2). |
申し込み: | WB IHC |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
反応性: | Human, Mouse |
ポジティブサンプル: | NCI-H460, U-251MG, MCF7, BT-474, Mouse liver, Mouse heart |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 406-645 of human DARS2 (NP_060592.2). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | ANRN WNSP VANF IMES QRLE LIRL METQ EEDV VLLT AGEH NKAC SLLG KLRL ECAD LLET RGVV LRDP TLFS FLWV VDFP LFLP KEEN PREL ESAH HPFT APHP SDIH LLYT EPKK ARSQ HYDL VLNG NEIG GGSI RIHN AELQ RYIL ATLL KEDV KMLS HLLQ ALDY GAPP HGGI ALGL DRLI CLVT GSPS IRDV IAFP KSFR GHDL MSNT PDSV PPEE LKPY HIRV SKPT DSKA ERAH |
遺伝子ID: | 55157 |
Uniprot: | Q6PI48 |
セルラーロケーション: | Mitochondrion matrix |
計算された分子量: | 73kDa |
観察された分子量: | 74kDa |
同義語: | DARS2, ASPRS, LBSL, MT-ASPRS |
バックグラウンド: | 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). |
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 |
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