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Merck
모든 사진(8)

주요 문서

HPA018997

Sigma-Aldrich

Anti-SLC25A13 antibody produced in rabbit

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

동의어(들):

Anti-Calcium-binding mitochondrial carrier protein Aralar2, Anti-Citrin, Anti-Mitochondrial aspartate glutamate carrier 2, Anti-Solute carrier family 25 member 13

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About This Item

UNSPSC 코드:
12352203
인간 단백질 도해서 번호:
NACRES:
NA.41

생물학적 소스

rabbit

Quality Level

결합

unconjugated

항체 형태

affinity isolated antibody

항체 생산 유형

primary antibodies

클론

polyclonal

제품 라인

Prestige Antibodies® Powered by Atlas Antibodies

양식

buffered aqueous glycerol solution

종 반응성

human

기술

immunoblotting: 0.04-0.4 μg/mL
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:50-1:200

면역원 서열

KVALTKRADPAELRTIFLKYASIEKNGEFFMSPNDFVTRYLNIFGESQPNPKTVELLSGVVDQTKDGLIS

UniProt 수납 번호

배송 상태

wet ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

일반 설명

The gene SLC25A13 (solute carrier family 25 member 13) is mapped to human chromosome 7q21.3. It contains EF-hand Ca2+-binding motifs and transmembrane domains. The protein localizes in the mitochondrial inner membrane. It is widely expressed with strong expression in liver. SLC25A13 is popularly called as citrin.

면역원

Calcium-binding mitochondrial carrier protein Aralar2 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.

생화학적/생리학적 작용

SLC25A13 (solute carrier family 25 member 13) is a Ca2+-binding mitochondrial aspartate/glutamate carrier and is crucial for the urea cycle and the aspartate/malate NADH (nicotinamide adenine dinucleotide) shuttle. Mutations in SLC25A13 cause neonatal intrahepatic cholestasis due to citrin deficiency (NICCD) in newborns, failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD) in older children and type II citrullinemia in adults.

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:
  • 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 APREST74829

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

면책조항

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

개인 보호 장비

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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시험 성적서(COA)

Lot/Batch Number

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문서 라이브러리 방문

D M Avdjieva-Tzavella et al.
Genetic counseling (Geneva, Switzerland), 25(3), 271-276 (2014-11-05)
Citrin deficiency is an autosomal recessive disorder caused by mutations in the SLC25A13 gene and has three phenotypes: neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) in newborns, failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD) in older
L Palmieri et al.
The EMBO journal, 20(18), 5060-5069 (2001-09-22)
The mitochondrial aspartate/glutamate carrier catalyzes an important step in both the urea cycle and the aspartate/malate NADH shuttle. Citrin and aralar1 are homologous proteins belonging to the mitochondrial carrier family with EF-hand Ca(2+)-binding motifs in their N-terminal domains. Both proteins
K Kobayashi et al.
Nature genetics, 22(2), 159-163 (1999-06-16)
Citrullinaemia (CTLN) is an autosomal recessive disease caused by deficiency of argininosuccinate synthetase (ASS). Adult-onset type II citrullinaemia (CTLN2) is characterized by a liver-specific ASS deficiency with no abnormalities in hepatic ASS mRNA or the gene ASS (refs 1-17). CTLN2
Rui Chen et al.
World journal of gastroenterology, 19(28), 4545-4551 (2013-08-01)
To investigate the differences in the mutation spectra of the SLC25A13 gene mutations from specific regions of China. Genetic analyses of SLC25A13 mutations were performed in 535 patients with neonatal intrahepatic cholestasis from our center over eight years. Unrelated infants

Global Trade Item Number

SKUGTIN
HPA018997-25UL4061842866601
HPA018997-100UL4061836312305

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