Signal Transduction Antibodies 1

Anti-SLC25A13 Antibody (CAB12557)

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SKU:
CAB12557
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Signal Transduction

Description

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Antibody Name:Anti-SLC25A13 Antibody
Antibody SKU:CAB12557
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC IF
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human SLC25A13 (NP_001153682.1).
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:Human
Positive Samples:MCF7, SW480, HepG2, HeLa
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human SLC25A13 (NP_001153682.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MAAA KVAL TKRA DPAE LRTI FLKY ASIE KNGE FFMS PNDF VTRY LNIF GESQ PNPK TVEL LSGV VDQT KDGL ISFQ EFVA FESV LCAP DALF MVAF QLFD KAGK GEVT FEDV KQVF GQTT IHQH IPFN WDSE FVQL HFGK ERKR HLTY AEFT QFLL EIQL EHAK QAFV QRDN ARTG RVTA IDFR DIMV TIRP HVLT PFVE ECLV AAAG GTTS HQVS FSYF NGFN SLLN NMEL IRKI YSTL AGTR KDVE VTKE EFVL AAQK FGQV TPME VDIL FQLA DLYE PRGR MTLA DIER IAPL EEGT
Gene ID:10165
Uniprot:Q9UJS0
Cellular Location:Mitochondrion inner membrane, Multi-pass membrane protein
Calculated MW:74kDa
Observed MW:74kDa
Synonyms:SLC25A13, ARALAR2, CITRIN, CTLN2
Background:This gene is a member of the mitochondrial carrier family. The encoded protein contains four EF-hand Ca(2+) binding motifs in the N-terminal domain, and localizes to mitochondria. The protein catalyzes the exchange of aspartate for glutamate and a proton across the inner mitochondrial membrane, and is stimulated by calcium on the external side of the inner mitochondrial membrane. Mutations in this gene result in citrullinemia, type II. Multiple transcript variants encoding different isoforms have been found for this gene.
UniProt Protein Function:SLC25A13: Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle. Defects in SLC25A13 are the cause of citrullinemia type 2 (CTLN2). Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN2 is characterized by neuropsychiatric symptoms including abnormal behaviors, loss of memory, seizures and coma. Death can result from brain edema. Onset is sudden and usually between the ages of 20 and 50 years. Defects in SLC25A13 are the cause of neonatal intrahepatic cholestasis due to citrin deficiency (NICCD). NICCD is a form of citrullinemia type 2 with neonatal onset. NICCD is characterized by suppression of the bile flow, hepatic fibrosis, low birth weight, growth retardation, hypoproteinemia, variable liver dysfunction. NICCD is generally not severe and symptoms disappear by one year of age with an appropriate diet. Years or even decades later, however, some individuals develop the characteristic features of citrullinemia type 2 with neuropsychiatric symptoms. Belongs to the mitochondrial carrier family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Mitochondrial; Membrane protein, multi-pass; Membrane protein, integral; Transporter, SLC family; Transporter

Chromosomal Location of Human Ortholog: 7q21.3

Cellular Component: mitochondrion; integral to plasma membrane; mitochondrial inner membrane

Molecular Function:L-aspartate transmembrane transporter activity; transporter activity; calcium ion binding; L-glutamate transmembrane transporter activity

Biological Process: L-glutamate transport; transport; ATP biosynthetic process; carbohydrate metabolic process; cellular respiration; glucose metabolic process; pathogenesis; aspartate transport; malate-aspartate shuttle; response to calcium ion; gluconeogenesis

Disease: Citrullinemia, Type Ii, Adult-onset; Citrullinemia, Type Ii, Neonatal-onset

NCBI Summary:This gene is a member of the mitochondrial carrier family. The encoded protein contains four EF-hand Ca(2+) binding motifs in the N-terminal domain, and localizes to mitochondria. The protein catalyzes the exchange of aspartate for glutamate and a proton across the inner mitochondrial membrane, and is stimulated by calcium on the external side of the inner mitochondrial membrane. Mutations in this gene result in citrullinemia, type II. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2009]
UniProt Code:Q9UJS0
NCBI GenInfo Identifier:13124095
NCBI Gene ID:10165
NCBI Accession:Q9UJS0.2
UniProt Related Accession:Q9UJS0
Molecular Weight:
NCBI Full Name:Calcium-binding mitochondrial carrier protein Aralar2
NCBI Synonym Full Names:solute carrier family 25 member 13
NCBI Official Symbol:SLC25A13  
NCBI Official Synonym Symbols:CTLN2; CITRIN; ARALAR2  
NCBI Protein Information:calcium-binding mitochondrial carrier protein Aralar2
UniProt Protein Name:Calcium-binding mitochondrial carrier protein Aralar2
UniProt Synonym Protein Names:Citrin; Mitochondrial aspartate glutamate carrier 2; Solute carrier family 25 member 13
Protein Family:COX assembly mitochondrial protein
UniProt Gene Name:SLC25A13  
UniProt Entry Name:CMC2_HUMAN
Anti-SLC25A13 Antibody (CAB12557)Western blot analysis of extracts of various cell lines, using SLC25A13 antibody (CAB12557) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
Anti-SLC25A13 Antibody (CAB12557)Immunohistochemistry of paraffin-embedded human lung cancer using SLC25A13 antibody (CAB12557) at dilution of 1:100 (40x lens).
Anti-SLC25A13 Antibody (CAB12557)Immunohistochemistry of paraffin-embedded human colon carcinoma using SLC25A13 antibody (CAB12557) at dilution of 1:100 (40x lens).
Anti-SLC25A13 Antibody (CAB12557)Immunofluorescence analysis of U2OS cells using SLC25A13 antibody (CAB12557). Blue: DAPI for nuclear staining.
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