![[Feedback]](/icons/banner/feedbackACT.gif){kind=icon}
![[For Subscribers]](/icons/banner/subscriberACT.gif){kind=icon}
![[Archive]](/icons/banner/archiveACT.gif){kind=icon}
![[Search]](/icons/banner/searchACT.gif){kind=icon}
![[Contents]](/icons/banner/tocACT.gif){kind=icon}
|
|
|
|
|
||
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.
This article has been cited by other articles:
|
|
 |
|
  S. Broer Amino Acid Transport Across Mammalian Intestinal and Renal Epithelia Physiol Rev, January 1, 2008; 88(1): 249 - 286. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Font-Llitjos, L. Feliubadalo, M. Espino, R. Cleries, S. Manas, I. M. Frey, S. Puertas, G. Colell, S. Palomo, J. Aranda, et al. Slc7a9 knockout mouse is a good cystinuria model for antilithiasic pharmacological studies Am J Physiol Renal Physiol, September 1, 2007; 293(3): F732 - F740. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. K. Grimble Adverse Gastrointestinal Effects of Arginine and Related Amino Acids J. Nutr., June 1, 2007; 137(6): 1693S - 1701S. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Rotmann, A. Simon, U. Martine, A. Habermeier, and E. I. Closs Activation of classical protein kinase C decreases transport via systems y+ and y+L Am J Physiol Cell Physiol, June 1, 2007; 292(6): C2259 - C2268. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. M. Malakauskas, H. Quan, T. A. Fields, S. J. McCall, M.-J. Yu, W. M. Kourany, C. W. Frey, and T. H. Le Aminoaciduria and altered renal expression of luminal amino acid transporters in mice lacking novel gene collectrin Am J Physiol Renal Physiol, February 1, 2007; 292(2): F533 - F544. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Moret, M. H. Dave, N. Schulz, J. X. Jiang, F. Verrey, and C. A. Wagner Regulation of renal amino acid transporters during metabolic acidosis Am J Physiol Renal Physiol, February 1, 2007; 292(2): F555 - F566. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. Fernandez, M. Jimenez-Vidal, M. Calvo, A. Zorzano, F. Tebar, M. Palacin, and J. Chillaron The Structural and Functional Units of Heteromeric Amino Acid Transporters: THE HEAVY SUBUNIT rBAT DICTATES OLIGOMERIZATION OF THE HETEROMERIC AMINO ACID TRANSPORTERS J. Biol. Chem., September 8, 2006; 281(36): 26552 - 26561. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Romeo, M. H. Dave, D. Bacic, Z. Ristic, S. M. R. Camargo, J. Loffing, C. A. Wagner, and F. Verrey Luminal kidney and intestine SLC6 amino acid transporters of B0AT-cluster and their tissue distribution in Mus musculus Am J Physiol Renal Physiol, February 1, 2006; 290(2): F376 - F383. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Castro-Fernandez, G. Maya-Nunez, and P. M. Conn Beyond the Signal Sequence: Protein Routing in Health and Disease Endocr. Rev., June 1, 2005; 26(4): 479 - 503. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Veljkovic, A. Bacconi, A. Stetak, A. Hajnal, S. Stasiuk, P. J. Skelly, I. Forster, C. B. Shoemaker, and F. Verrey Aromatic Amino Acid Transporter AAT-9 of Caenorhabditis elegans Localizes to Neurons and Muscle Cells J. Biol. Chem., November 19, 2004; 279(47): 49268 - 49273. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. C. Bridges and R. K. Zalups Homocysteine, System b0,+ and the Renal Epithelial Transport and Toxicity of Inorganic Mercury Am. J. Pathol., October 1, 2004; 165(4): 1385 - 1394. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Gasol, M. Jimenez-Vidal, J. Chillaron, A. Zorzano, and M. Palacin Membrane Topology of System Xc- Light Subunit Reveals a Re-entrant Loop with Substrate-restricted Accessibility J. Biol. Chem., July 23, 2004; 279(30): 31228 - 31236. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. H. Dave, N. Schulz, M. Zecevic, C. A. Wagner, and F. Verrey Expression of heteromeric amino acid transporters along the murine intestine J. Physiol., July 15, 2004; 558(2): 597 - 610. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. C. Bridges, C. Bauch, F. Verrey, and R. K. Zalups Mercuric Conjugates of Cysteine Are Transported by the Amino Acid Transporter System b0,+: Implications of Molecular Mimicry J. Am. Soc. Nephrol., March 1, 2004; 15(3): 663 - 673. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Veljkovic, S. Stasiuk, P. J. Skelly, C. B. Shoemaker, and F. Verrey Functional Characterization of Caenorhabditis elegans Heteromeric Amino Acid Transporters J. Biol. Chem., February 27, 2004; 279(9): 7655 - 7662. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Feliubadalo, M. L. Arbones, S. Manas, J. Chillaron, J. Visa, M. Rodes, F. Rousaud, A. Zorzano, M. Palacin, and V. Nunes Slc7a9-deficient mice develop cystinuria non-I and cystine urolithiasis Hum. Mol. Genet., September 1, 2003; 12(17): 2097 - 2108. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Fernandez, D. Torrents, J. Chillaron, R. Martin del Rio, A. Zorzano, and M. Palacin Basolateral LAT-2 Has a Major Role in the Transepithelial Flux of L-Cystine in the Renal Proximal Tubule Cell Line OK J. Am. Soc. Nephrol., April 1, 2003; 14(4): 837 - 847. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Bauch, N. Forster, D. Loffing-Cueni, V. Summa, and F. Verrey Functional Cooperation of Epithelial Heteromeric Amino Acid Transporters Expressed in Madin-Darby Canine Kidney Cells J. Biol. Chem., January 3, 2003; 278(2): 1316 - 1322. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Fernandez, M. Carrascal, F. Rousaud, J. Abian, A. Zorzano, M. Palacin, and J. Chillaron rBAT-b0,+AT heterodimer is the main apical reabsorption system for cystine in the kidney Am J Physiol Renal Physiol, September 1, 2002; 283(3): F540 - F548. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K.-i. Hosoya, M. Tomi, S. Ohtsuki, H. Takanaga, S. Saeki, Y. Kanai, H. Endou, M. Naito, T. Tsuruo, and T. Terasaki Enhancement of L-Cystine Transport Activity and Its Relation to xCT Gene Induction at the Blood-Brain Barrier by Diethyl Maleate Treatment J. Pharmacol. Exp. Ther., July 1, 2002; 302(1): 225 - 231. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Bauch and F. Verrey Apical heterodimeric cystine and cationic amino acid transporter expressed in MDCK cells Am J Physiol Renal Physiol, July 1, 2002; 283(1): F181 - F189. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Matsuo, Y. Kanai, J. Y. Kim, A. Chairoungdua, D. K. Kim, J. Inatomi, Y. Shigeta, H. Ishimine, S. Chaekuntode, K. Tachampa, et al. Identification of a Novel Na+-independent Acidic Amino Acid Transporter with Structural Similarity to the Member of a Heterodimeric Amino Acid Transporter Family Associated with Unknown Heavy Chains J. Biol. Chem., May 31, 2002; 277(23): 21017 - 21026. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Tomi, K.-i. Hosoya, H. Takanaga, S. Ohtsuki, and T. Terasaki Induction of xCT Gene Expression and L-Cystine Transport Activity by Diethyl Maleate at the Inner Blood-Retinal Barrier Invest. Ophthalmol. Vis. Sci., March 1, 2002; 43(3): 774 - 779. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Chillaron, R. Roca, A. Valencia, A. Zorzano, and M. Palacin Heteromeric amino acid transporters: biochemistry, genetics, and physiology Am J Physiol Renal Physiol, December 1, 2001; 281(6): F995 - F1018. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. C. H. Friesema, R. Docter, E. P. C. M. Moerings, F. Verrey, E. P. Krenning, G. Hennemann, and T. J. Visser Thyroid Hormone Transport by the Heterodimeric Human System L Amino Acid Transporter Endocrinology, October 1, 2001; 142(10): 4339 - 4348. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. A. Wagner, F. Lang, and S. Broer Function and structure of heterodimeric amino acid transporters Am J Physiol Cell Physiol, October 1, 2001; 281(4): C1077 - C1093. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Hennemann, R. Docter, E. C. H. Friesema, M. de Jong, E. P. Krenning, and T. J. Visser Plasma Membrane Transport of Thyroid Hormones and Its Role in Thyroid Hormone Metabolism and Bioavailability Endocr. Rev., August 1, 2001; 22(4): 451 - 476. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Font, L. Feliubadalo, X. Estivill, V. Nunes, E. Golomb, Y. Kreiss, E. Pras, L. Bisceglia, A. P. d'Adamo, L. Zelante, et al. Functional analysis of mutations in SLC7A9, and genotype-phenotype correlation in non-Type I cystinuria Hum. Mol. Genet., February 1, 2001; 10(4): 305 - 316. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Chairoungdua, Y. Kanai, H. Matsuo, J. Inatomi, D. K. Kim, and H. Endou Identification and Characterization of a Novel Member of the Heterodimeric Amino Acid Transporter Family Presumed to be Associated with an Unknown Heavy Chain J. Biol. Chem., December 21, 2001; 276(52): 49390 - 49399. [Abstract] [Full Text] [PDF]
|
|
