Calcium-binding mitochondrial carrier protein Aralar2 (SLC25A13), ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C.

We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team

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Small volumes of SLC25A13 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS7208868 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Calcium-binding mitochondrial carrier protein Aralar2 (SLC25A13) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing SLC25A13. The ELISA analytical biochemical technique of the MBS7208868 kit is based on SLC25A13 antibody-SLC25A13 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect SLC25A13 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, SLC25A13. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Neurobiology

74,304 Da

solute carrier family 25 (aspartate/glutamate carrier), member 13

calcium-binding mitochondrial carrier protein Aralar2; mitochondrial aspartate glutamate carrier 2; solute carrier family 25, member 13 (citrin)

Calcium-binding mitochondrial carrier protein Aralar2

ARALAR2  [Similar Products]

CMC2_HUMAN

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]

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, integral; Transporter; Transporter, SLC family; Membrane protein, multi-pass

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; cellular respiration; carbohydrate metabolic process; glucose metabolic process; pathogenesis; aspartate transport; malate-aspartate shuttle; response to calcium ion; gluconeogenesis

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

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