DARS2 Antibody (PACO46662)
제품 코드를 사용하여 Assay Genie 메인 사이트를 통해 주문합니다.
주문- SKU:
- PACO46662
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | DARS2 Antibody (PACO46662) |
Antibody SKU: | PACO46662 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:1000-1:5000, IHC:1:20-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Aspartate--tRNA ligase, mitochondrial protein (489-628AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western blot. All lanes: DARS2 antibody at 10µg/ml + MCF-7 whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 74 kDa. Observed band size: 74 kDa.. | |
Immunohistochemistry of paraffin-embedded human kidney tissue using PACO46662 at dilution of 1:100. |
Background: | 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 |
Synonyms: | 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 |