Sodium-dependent neutral amino acid transporter B(0)AT1; Solute carrier family 6 member 19; System B(0) neutral amino acid transporter AT1; SLC6A19; B0AT1

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

Polyclonal

Rabbit Ig

Rabbit

This SLC6A19 antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 544-575 amino acids from human SLC6A19.

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Maintain refrigerated at 2-8 degree C for up to 2 weeks. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-SLC6A19 antibody 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.

Transporter that mediates epithelial resorption of neutral amino acids across the apical membrane of epithelial cells in the kidney and intestine. It appears that leucine is the preferred substrate, but all large neutral non-aromatic L-amino acids bind to this transporter. Uptake of leucine is sodium- dependent. In contrast to other members of the neurotransmitter transporter family, does not appear to be chloride-dependent (By similarity).

New Antibodies; Primary Antibodies; Neuroscience

Western Blot (WB), ELISA (EIA)

WB:~~1:2000

Anti-SLC6A19 Antibody (C-Term) at 1:2000 dilution + 293 whole cell lysate
Lysates/proteins at 20 ug per lane.
Secondary
Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution.
Predicted band size : 71 kDa
Blocking/Dilution buffer: 5% NFDM/TBST.

71110

solute carrier family 6 member 19

sodium-dependent neutral amino acid transporter B(0)AT1

Sodium-dependent neutral amino acid transporter B(0)AT1

B0AT1??[Similar Products]

S6A19_HUMAN

This gene encodes a system B(0) transmembrane protein that actively transports most neutral amino acids across the apical membrane of epithelial cells. Mutations in this gene result in Hartnup disorder. [provided by RefSeq, Jul 2008]

SLC6A19: Transporter that mediates epithelial resorption of neutral amino acids across the apical membrane of epithelial cells in the kidney and intestine. It appears that leucine is the preferred substrate, but all large neutral non-aromatic L-amino acids bind to this transporter. Uptake of leucine is sodium- dependent. In contrast to other members of the neurotransmitter transporter family, does not appear to be chloride-dependent. Defects in SLC6A19 are a cause of Hartnup disorder (HND). HND is an autosomal recessive abnormality of renal and gastrointestinal neutral amino acid transport noted for its clinical variability. First described in 1956, HND is characterized by increases in the urinary and intestinal excretion of neutral amino acids. Individuals with typical Hartnup aminoaciduria may be asymptomatic, some develop a photosensitive pellagra-like rash, attacks of cerebellar ataxia and other neurological or psychiatric features. Although the definition of HND was originally based on clinical and biochemical abnormalities, its marked clinical heterogeneity has led to it being known as a disorder with a consistent pathognomonic neutral hyperaminoaciduria. Defects in SLC6A19 may be a cause of hyperglycinuria (HG). It is a condition characterized by excess of glycine in the urine. In some cases it is associated with renal colic and renal oxalate stones. SLC6A19 deficiency combined with haploinsufficiency of SLC6A20 or partially inactivating mutations in SLC36A2, can be responsible for hyperglycinuria. Defects in SLC6A19 may be a cause of iminoglycinuria (IG). It is a disorder of renal tubular reabsorption of glycine and imino acids (proline and hydroxyproline), marked by excessive levels of all three substances in the urine. SLC6A19 deficiency combined with haploinsufficiency of SLC6A20 or partially inactivating mutations in SLC36A2, can be responsible for iminoglycinuria. Additional polymorphisms and mutations in SLC6A18 can contribute to the IG phenotype in some families. Belongs to the sodium:neurotransmitter symporter (SNF) (TC 2.A.22) family. SLC6A19 subfamily.

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

Chromosomal Location of Human Ortholog: 5p15.33

Cellular Component: brush border membrane; integral to plasma membrane; plasma membrane

Molecular Function: amino acid transmembrane transporter activity; neurotransmitter:sodium symporter activity; neutral amino acid transmembrane transporter activity

Biological Process: amino acid transport; neurotransmitter transport; neutral amino acid transport; response to nutrient

Disease: Hartnup Disorder; Hyperglycinuria; Iminoglycinuria

Kleta R.,et al.Nat. Genet. 36:999-1002 (2004). Seow H.F.,et al.Nat. Genet. 36:1003-1007 (2004). Ota T.,et al.Nat. Genet. 36:40-45 (2004). Mural R.J.,et al.Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases. Broer S.,et al.J. Clin. Invest. 118:3881-3892 (2008).

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