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Mutations of the glycoprotein rBAT cause cystinuria type I, an autosomal recessive failure of dibasic amino acid transport (b(0,+) type) across luminal membranes of intestine and kidney cells. Here we identify the permease-like protein b(0,+)AT as the catalytic subunit that associates by a disulfide bond with rBAT to form a hetero-oligomeric b(0,+) amino acid transporter complex. We demonstrate its b(0,+)-type amino acid transport kinetics using a heterodimeric fusion construct and show its luminal brush border localization in kidneyproximal tubule. These biochemical, transport, and localization characteristics as well as the chromosomal localization on 19q support the notion that the b(0,+)AT protein is the product of the gene defective in non-type I cystinuria.
Bertran,
Expression cloning of a human renal cDNA that induces high affinity transport of L-cystine shared with dibasic amino acids in Xenopus oocytes.
1993, Pubmed,
Xenbase
Bertran,
Expression cloning of a human renal cDNA that induces high affinity transport of L-cystine shared with dibasic amino acids in Xenopus oocytes.
1993,
Pubmed
,
Xenbase Bertran,
Expression cloning of a cDNA from rabbit kidney cortex that induces a single transport system for cystine and dibasic and neutral amino acids.
1992,
Pubmed
,
Xenbase Bisceglia,
Localization, by linkage analysis, of the cystinuria type III gene to chromosome 19q13.1.
1997,
Pubmed Borsani,
SLC7A7, encoding a putative permease-related protein, is mutated in patients with lysinuric protein intolerance.
1999,
Pubmed Busch,
Opposite directed currents induced by the transport of dibasic and neutral amino acids in Xenopus oocytes expressing the protein rBAT.
1994,
Pubmed
,
Xenbase Calonge,
Cystinuria caused by mutations in rBAT, a gene involved in the transport of cystine.
1994,
Pubmed
,
Xenbase Chillarón,
Obligatory amino acid exchange via systems bo,+-like and y+L-like. A tertiary active transport mechanism for renal reabsorption of cystine and dibasic amino acids.
1996,
Pubmed
,
Xenbase Coady,
Electrogenic amino acid exchange via the rBAT transporter.
1994,
Pubmed
,
Xenbase Estévez,
The amino acid transport system y+L/4F2hc is a heteromultimeric complex.
1998,
Pubmed
,
Xenbase Foreman,
Transport interactions of cystine and dibasic amino acids in isolated rat renal tubules.
1980,
Pubmed Furriols,
rBAT, related to L-cysteine transport, is localized to the microvilli of proximal straight tubules, and its expression is regulated in kidney by development.
1993,
Pubmed
,
Xenbase Geering,
A role for the beta-subunit in the expression of functional Na+-K+-ATPase in Xenopus oocytes.
1989,
Pubmed
,
Xenbase Kanai,
Expression of mRNA (D2) encoding a protein involved in amino acid transport in S3 proximal tubule.
1992,
Pubmed
,
Xenbase Kanai,
Expression cloning and characterization of a transporter for large neutral amino acids activated by the heavy chain of 4F2 antigen (CD98).
1998,
Pubmed
,
Xenbase Loffing,
Thiazide treatment of rats provokes apoptosis in distal tubule cells.
1996,
Pubmed Mannion,
The light chain of CD98 is identified as E16/TA1 protein.
1998,
Pubmed Mao,
A string of enzymes, purification and characterization of a fusion protein comprising the four subunits of the glucose phosphotransferase system of Escherichia coli.
1995,
Pubmed Mastroberardino,
Amino-acid transport by heterodimers of 4F2hc/CD98 and members of a permease family.
1998,
Pubmed
,
Xenbase Mosckovitz,
Membrane topology of the rat kidney neutral and basic amino acid transporter.
1994,
Pubmed
,
Xenbase Nakamura,
4F2 (CD98) heavy chain is associated covalently with an amino acid transporter and controls intracellular trafficking and membrane topology of 4F2 heterodimer.
1999,
Pubmed
,
Xenbase Palacín,
Molecular biology of mammalian plasma membrane amino acid transporters.
1998,
Pubmed Palacín,
Role of the b(o,+)-like amino acid-transport system in the renal reabsorption of cystine and dibasic amino acids.
1996,
Pubmed Pfeiffer,
Functional heterodimeric amino acid transporters lacking cysteine residues involved in disulfide bond.
1998,
Pubmed
,
Xenbase Pfeiffer,
Amino acid transport of y+L-type by heterodimers of 4F2hc/CD98 and members of the glycoprotein-associated amino acid transporter family.
1999,
Pubmed
,
Xenbase Pras,
Biochemical and clinical studies in Libyan Jewish cystinuria patients and their relatives.
1998,
Pubmed Prasad,
Human LAT1, a subunit of system L amino acid transporter: molecular cloning and transport function.
1999,
Pubmed Puoti,
Novel isoforms of the beta and gamma subunits of the Xenopus epithelial Na channel provide information about the amiloride binding site and extracellular sodium sensing.
1997,
Pubmed
,
Xenbase Quackenbush,
Differential localization within human kidney of five membrane proteins expressed on acute lymphoblastic leukemia cells.
1986,
Pubmed Riahi-Esfahani,
Comparative study of the uptake of L-cysteine and L-cystine in the renal proximal tubule.
1995,
Pubmed Rosenberg,
Cystinuria: biochemical evidence for three genetically distinct diseases.
1966,
Pubmed Rossier,
LAT2, a new basolateral 4F2hc/CD98-associated amino acid transporter of kidney and intestine.
1999,
Pubmed
,
Xenbase Schafer,
Transport of L-cystine in isolated perfused proximal straight tubules.
1984,
Pubmed Silbernagl,
The renal handling of amino acids and oligopeptides.
1988,
Pubmed Tate,
Expression cloning of a Na(+)-independent neutral amino acid transporter from rat kidney.
1992,
Pubmed
,
Xenbase Torrents,
Identification of SLC7A7, encoding y+LAT-1, as the lysinuric protein intolerance gene.
1999,
Pubmed
,
Xenbase Torrents,
Identification and characterization of a membrane protein (y+L amino acid transporter-1) that associates with 4F2hc to encode the amino acid transport activity y+L. A candidate gene for lysinuric protein intolerance.
1998,
Pubmed
,
Xenbase Van Winkle,
Amino acid transport in developing animal oocytes and early conceptuses.
1988,
Pubmed Wang,
Oligomeric structure of a renal cystine transporter: implications in cystinuria.
1995,
Pubmed
,
Xenbase Wartenfeld,
Molecular analysis of cystinuria in Libyan Jews: exclusion of the SLC3A1 gene and mapping of a new locus on 19q.
1997,
Pubmed Wells,
Cloning of a rat kidney cDNA that stimulates dibasic and neutral amino acid transport and has sequence similarity to glucosidases.
1992,
Pubmed
,
Xenbase Yan,
Distribution of mRNA of a Na(+)-independent neutral amino acid transporter cloned from rat kidney and its expression in mammalian tissues and Xenopus laevis oocytes.
1992,
Pubmed
,
Xenbase
