Mouse anti-Human SLC25A13 Monoclonal Antibody | anti-SLC25A13 antibody

SLC25A13 (Calcium-binding Mitochondrial Carrier Protein Aralar2, Mitochondrial Aspartate Glutamate Carrier 2, Solute Carrier Family 25 Member 13, Citrin, ARALAR2) (Biotin)

Cat. Num. AAA6144403

• Gene Names: SLC25A13; CTLN2; NICCD; CITRIN; ARALAR2
• Reactivity: Human
• Applications: ELISA, Immunofluorescence, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: SLC25A13; Monoclonal Antibody; SLC25A13 (Calcium-binding Mitochondrial Carrier Protein Aralar2; Mitochondrial Aspartate Glutamate Carrier 2; Solute Carrier Family 25 Member 13; Citrin; ARALAR2) (Biotin); anti-SLC25A13 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 4F4
• Specificity: Recognizes human SLC25A13.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
• Sequence Length: 3141

• Applicable Applications for anti-SLC25A13 antibody: ELISA (EIA), Immunofluorescence (IF), Western Blot (WB)
• Application Notes: IF: 10ug/ml; Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa2-80 from SLC25A13 (NP_055066) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: AAAKVALTKRADPAELRTIFLKYASIEKNGEFFMSPNDFVTRYLNIFGESQPNPKTVELLSGVVDQTKDGLISFQEFVA
• Conjugate: Biotin
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (34.8kD).)

Western Blot (WB)

(Western Blot analysis of SLC25A13 expression in transfected 293T cell line by SLC25A13 monoclonal antibody Lane 1: SLC25A13 transfected lysate (74.2kD). Lane 2: Non-transfected lysate.)

Immunofluorescence (IF)

(Immunofluorescence of monoclonal antibody to SLC25A13 on HepG2 cell. [antibody concentration 10ug/ml])

Testing Data

(Detection limit for recombinant GST tagged SLC25A13 is ~0.3ng/ml as a capture antibody.)

Western Blot (WB)

(Western blot analysis of SLC25A13 over-expressed 293 cell line, cotransfected with SLC25A13 Validated Chimera RNAi (Lane 2) or non-transfected control (Lane 1). Blot probed with SLC25A13 monoclonal antibody GAPDH (36.1kD) used as specificity and loading control.)

Western Blot (WB)

(SLC25A13 monoclonal antibody Western Blot analysis of SLC25A13 expression in HepG2)

Product Categories/Family for anti-SLC25A13 antibody

Antibodies; Ion Channel

NCBI and Uniprot Product Information

• NCBI GI #: 1677530170
• NCBI GeneID: 10165
• NCBI Accession #: NM_014251.3
• NCBI GenBank Nucleotide #: NM_014251
• UniProt Accession #: Q9UJS0
• NCBI Official Full Name: Homo sapiens solute carrier family 25 member 13 (SLC25A13), transcript variant 2, mRNA
• NCBI Official Synonym Full Names: solute carrier family 25 member 13
• NCBI Official Symbol: SLC25A13
• NCBI Official Synonym Symbols: CTLN2; NICCD; CITRIN; ARALAR2
• NCBI Protein Information: calcium-binding mitochondrial carrier protein Aralar2
• UniProt Protein Name: Calcium-binding mitochondrial carrier protein Aralar2
• Protein Family: Calcium-binding mitochondrial carrier protein
• UniProt Gene Name: SLC25A13
• UniProt Synonym Gene Names: ARALAR2
• UniProt Entry Name: CMC2_HUMAN

NCBI Description

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 Description

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.

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