추천 제품
생물학적 소스
rabbit
결합
unconjugated
항체 형태
affinity isolated antibody
항체 생산 유형
primary antibodies
클론
polyclonal
제품 라인
Prestige Antibodies® Powered by Atlas Antibodies
형태
buffered aqueous glycerol solution
종 반응성
human
향상된 검증
independent
Learn more about Antibody Enhanced Validation
기술
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:20-1:50
면역원 서열
EVTLCGWIQYRRQNTFLVLRDFDGLVQVIIPQDESAASVKKILCEAPVESVVQVSGTVISRPAGQENPKMPTGEIEIKVKTAELLNACKKLPFEIKNFVKK
UniProt 수납 번호
배송 상태
wet ice
저장 온도
−20°C
타겟 번역 후 변형
unmodified
유전자 정보
human ... DARS2(55157)
일반 설명
Aspartyl-tRNA synthetase 2, mitochondrial (DARS2) is a homodimeric enzyme. The gene encoding it is localized on human chromosome 1q25.1.
면역원
Aspartyl-tRNA synthetase, mitochondrial Precursor recombinant protein epitope signature tag (PrEST)
애플리케이션
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
생화학적/생리학적 작용
Aspartyl-tRNA synthetase 2, mitochondrial (DARS2) has a crucial role in the attachment of aspartate to the right mitochondrial transfer RNA. Mutations in the gene encoding it have been associated with leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate (LBSL).
특징 및 장점
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
결합
Corresponding Antigen APREST76658
물리적 형태
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
법적 정보
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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Storage Class Code
10 - Combustible liquids
WGK
WGK 1
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
A novel homozygous mutation of DARS2 may cause a severe LBSL variant.
Miyake N
Clinical Genetics, 80(3), 293-296 (2011)
Pathogenic mutations causing LBSL affect mitochondrial aspartyl-tRNA synthetase in diverse ways.
van Berge L
The Biochemical Journal, 450(2), 345-350 (2013)
DARS-associated leukoencephalopathy can mimic a steroid-responsive neuroinflammatory disorder.
Wolf NI, et.l
Neurology, 84(3), 226-230 (2015)
Laura van Berge et al.
Brain : a journal of neurology, 137(Pt 4), 1019-1029 (2014-02-26)
Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation is a disorder caused by recessive mutations in the gene DARS2, which encodes mitochondrial aspartyl-tRNA synthetase. Recent observations indicate that the phenotypic range of the disease is much wider than
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