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. 2000 Nov 1;351(Pt 3):677–682.

Cysteine residues in the C-terminus of the neutral- and basic-amino-acid transporter heavy-chain subunit contribute to functional properties of the system b(0,+)-type amino acid transporter.

G J Peter 1, T B Panova 1, G R Christie 1, P M Taylor 1
PMCID: PMC1221407  PMID: 11042122

Abstract

The neutral- and basic-amino-acid-transport glycoprotein NBAT (rBAT, D2) expressed in renal and jejunal brush-border membranes interacts with the b(0,+)AT permease to produce a heteromeric transporter effecting amino acid and cystine absorption. NBAT mutations result in type I cystinuria. The b(0,+)AT permease is presumed to be the catalytic subunit, but we have been investigating the possibility that cysteine residues within the C-terminus of NBAT are also important for expression of transport function. NBAT mutants were produced with combinations of Cys(664/671/683)-->Ala substitutions. Mutants with Cys(664)-->Ala show decreased arginine and cystine transport and specifically lose sensitivity to inhibition of transport by the thiol-group reagent N-ethylmaleimide (NEM). We suggest that the C-terminus of NBAT may have a direct role in the mechanism of System b(0,+) transport (the major transport activity defective in type I cystinuria) and that Cys(664) of NBAT is the major target for NEM-induced inactivation of the transport mechanism.

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Selected References

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