PMID- 10588648
OWN - NLM
STAT- MEDLINE
DCOM- 20000214
LR  - 20181113
IS  - 1059-1524 (Print)
IS  - 1059-1524 (Linking)
VI  - 10
IP  - 12
DP  - 1999 Dec
TI  - Luminal heterodimeric amino acid transporter defective in cystinuria.
PG  - 4135-47
AB  - 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 kidney
      proximal 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.
FAU - Pfeiffer, R
AU  - Pfeiffer R
AD  - Institute of Physiology, University of Zurich, CH-8057 Zurich, Switzerland.
FAU - Loffing, J
AU  - Loffing J
FAU - Rossier, G
AU  - Rossier G
FAU - Bauch, C
AU  - Bauch C
FAU - Meier, C
AU  - Meier C
FAU - Eggermann, T
AU  - Eggermann T
FAU - Loffing-Cueni, D
AU  - Loffing-Cueni D
FAU - Kuhn, L C
AU  - Kuhn LC
FAU - Verrey, F
AU  - Verrey F
LA  - eng
SI  - GENBANK/AJ249198
SI  - GENBANK/AJ249199
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Mol Biol Cell
JT  - Molecular biology of the cell
JID - 9201390
RN  - 0 (Amino Acid Transport Systems)
RN  - 0 (Amino Acid Transport Systems, Basic)
RN  - 0 (Amino Acids)
RN  - 0 (Carrier Proteins)
RN  - 0 (Membrane Glycoproteins)
RN  - 0 (SLC7A9 protein, human)
RN  - 0 (Slc7a9 protein, mouse)
SB  - IM
MH  - Amino Acid Sequence
MH  - Amino Acid Transport Systems
MH  - *Amino Acid Transport Systems, Basic
MH  - Amino Acids/*metabolism
MH  - Animals
MH  - Biological Transport
MH  - Carrier Proteins/genetics/*metabolism
MH  - *Chromosomes, Human, Pair 19
MH  - Cloning, Molecular
MH  - Cystinuria/genetics/*metabolism
MH  - Fluorescent Antibody Technique
MH  - Humans
MH  - In Situ Hybridization
MH  - Kidney/metabolism/ultrastructure
MH  - Male
MH  - Membrane Glycoproteins/genetics/*metabolism
MH  - Mice
MH  - Microvilli/metabolism
MH  - Molecular Sequence Data
MH  - Oocytes/metabolism
MH  - Organ Specificity
MH  - Sequence Alignment
MH  - Xenopus laevis
PMC - PMC25748
EDAT- 1999/12/10 09:00
MHDA- 2000/02/19 09:00
CRDT- 1999/12/10 09:00
PHST- 1999/12/10 09:00 [pubmed]
PHST- 2000/02/19 09:00 [medline]
PHST- 1999/12/10 09:00 [entrez]
AID - 10.1091/mbc.10.12.4135 [doi]
PST - ppublish
SO  - Mol Biol Cell. 1999 Dec;10(12):4135-47. doi: 10.1091/mbc.10.12.4135.