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Vol. 10, Issue 12, 4135-4147, December 1999
and
Institutes of *Physiology and Mutations of the glycoprotein rBAT cause cystinuria type I, an
autosomal recessive failure of dibasic amino acid transport (b0,+ type) across luminal membranes of intestine and
kidney cells. Here we identify the permease-like protein
b0,+AT as the catalytic subunit that associates by a
disulfide bond with rBAT to form a hetero-oligomeric b0,+
amino acid transporter complex. We demonstrate its
b0,+-type amino acid transport kinetics using a
heterodimeric fusion construct and show its luminal brush border
localization in kidney proximal tubule. These biochemical, transport,
and localization characteristics as well as the chromosomal
localization on 19q support the notion that the b0,+AT
protein is the product of the gene defective in non-type I cystinuria.
Anatomy, University of
Zürich, CH-8057 Zürich, Switzerland; Swiss
Institute for Experimental Cancer Research, CH-1066 Epalinges,
Switzerland; and §Institute for Human Genetics, University
Hospital of the Rheinisch-Westfälische Technische Hochschule,
D-52074 Aachen, Germany
Corresponding author. E-mail
address: verrey{at}physiol.unizh.ch.
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