HPA018997
Anti-SLC25A13 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Sinonimo/i:
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
Prodotti consigliati
Origine biologica
rabbit
Coniugato
unconjugated
Forma dell’anticorpo
affinity isolated antibody
Tipo di anticorpo
primary antibodies
Clone
polyclonal
Nome Commerciale
Prestige Antibodies® Powered by Atlas Antibodies
Forma fisica
buffered aqueous glycerol solution
Reattività contro le specie
human
tecniche
immunoblotting: 0.04-0.4 μg/mL
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:50-1:200
Sequenza immunogenica
KVALTKRADPAELRTIFLKYASIEKNGEFFMSPNDFVTRYLNIFGESQPNPKTVELLSGVVDQTKDGLIS
N° accesso UniProt
Condizioni di spedizione
wet ice
Temperatura di conservazione
−20°C
modifica post-traduzionali bersaglio
unmodified
Informazioni sul gene
human ... SLC25A13(10165)
Descrizione generale
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.
Immunogeno
Calcium-binding mitochondrial carrier protein Aralar2 recombinant protein epitope signature tag (PrEST)
Applicazioni
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.
Azioni biochim/fisiol
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.
Caratteristiche e vantaggi
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.
Linkage
Corresponding Antigen APREST74829
Stato fisico
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Note legali
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Esclusione di responsabilità
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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Codice della classe di stoccaggio
10 - Combustible liquids
Classe di pericolosità dell'acqua (WGK)
WGK 1
Punto d’infiammabilità (°F)
Not applicable
Punto d’infiammabilità (°C)
Not applicable
Dispositivi di protezione individuale
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
Certificati d'analisi (COA)
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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
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
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
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