Cystic canal mutants in Caenorhabditis elegans are defective in the apical membrane domain of the renal (excretory) cell

被引:110
作者
Buechner, M [1 ]
Hall, DH
Bhatt, H
Hedgecock, EM
机构
[1] Johns Hopkins Univ, Dept Biol, Baltimore, MD 21218 USA
[2] Univ Kansas, Dept Mol Biosci, Lawrence, KS 66045 USA
[3] Albert Einstein Coll Med, Ctr C Elegans Anat, Bronx, NY 10461 USA
[4] Roche Inst Mol Biol, Dept Cell Biol, Nutley, NJ 07110 USA
关键词
renal epithelium; excretory canals; polycystic kidney disease; tubulogenesis; nematode;
D O I
10.1006/dbio.1999.9398
中图分类号
Q [生物科学];
学科分类号
07 ; 0710 ; 09 ;
摘要
The excretory cell extends a tubular process, or canal, along the basolateral surface of the epidermis to form the nematode renal epithelium. This cell can undergo normal tubulogenesis in isolated cell culture. Mutations in 12 genes cause excretory canal cysts in Caenorhabditis elegans. Genetic interactions, and their similar phenotypes, suggest these genes may encode functionally related proteins. Depending upon genotype and individual canal, defects range from focal cysts, flanked by normal width segments, to regional cysts involving the entire tubule. Oftentimes the enlarged regions are convoluted or partially septated. In mutants with very large cysts, renal function is measurably impaired. Based on histology and ultrastructure, canal cysts likely result from defects of the apical membrane domain. These mutants provide a model of tubulocystic disease without hyperplasia or basement membrane abnormalities. Similar apical mechanisms could regulate tubular morphology of vertebrate nephrons. (C) 1999 Academic Press.
引用
收藏
页码:227 / 241
页数:15
相关论文
共 73 条
  • [1] ANIMAL-MODELS OF POLYCYSTIC KIDNEY-DISEASE
    AZIZ, N
    [J]. BIOESSAYS, 1995, 17 (08) : 703 - 712
  • [2] BENNETT V, 1993, ANNU REV CELL BIOL, V9, P27, DOI 10.1146/annurev.cb.09.110193.000331
  • [3] BERNSTEIN J, 1990, CYSTIC KIDNEY, P147
  • [4] Immunolocalization of ion transport proteins in human autosomal dominant polycystic kidney epithelial cells
    Brill, SR
    Ross, KE
    Davidow, CJ
    Ye, M
    Grantham, JJ
    Caplan, MJ
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1996, 93 (19) : 10206 - 10211
  • [5] A P-GLYCOPROTEIN PROTECTS CAENORHABDITIS-ELEGANS AGAINST NATURAL TOXINS
    BROEKS, A
    JANSSEN, HWRM
    CALAFAT, J
    PLASTERK, RHA
    [J]. EMBO JOURNAL, 1995, 14 (09) : 1858 - 1866
  • [6] ANALYSIS OF THE GENOMIC SEQUENCE FOR THE AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY-DISEASE (PKD1) GENE PREDICTS THE PRESENCE OF A LEUCINE-RICH REPEAT
    BURN, TC
    CONNORS, TD
    DACKOWSKI, WR
    PETRY, LR
    VANRAAY, TJ
    MILLHOLLAND, JM
    VENET, M
    MILLER, G
    HAKIM, RM
    LANDES, GM
    KLINGER, KW
    FENG, Q
    ONUCHIC, LF
    WATNICK, T
    GERMINO, GG
    DOGGETT, NA
    [J]. HUMAN MOLECULAR GENETICS, 1995, 4 (04) : 575 - 582
  • [7] The structure of a PKD domain from polycystin-1: Implications for polycystic kidney disease
    Bycroft, M
    Bateman, A
    Clarke, J
    Hamill, SJ
    Sandford, R
    Thomas, RL
    Chothia, C
    [J]. EMBO JOURNAL, 1999, 18 (02) : 297 - 305
  • [8] Genome sequence of the nematode C-elegans:: A platform for investigating biology
    不详
    [J]. SCIENCE, 1998, 282 (5396) : 2012 - 2018
  • [9] Calvet JP, 1998, J NEPHROL, V11, P24
  • [10] ANISOSMOTIC CELL-VOLUME REGULATION - A COMPARATIVE VIEW
    CHAMBERLIN, ME
    STRANGE, K
    [J]. AMERICAN JOURNAL OF PHYSIOLOGY, 1989, 257 (02): : C159 - C173