PMID- 10200981
OWN - NLM
STAT- MEDLINE
DCOM- 19990714
LR  - 20181201
IS  - 0085-2538 (Print)
IS  - 0085-2538 (Linking)
VI  - 55
IP  - 4
DP  - 1999 Apr
TI  - New insights into the molecular pathophysiology of polycystic kidney disease.
PG  - 1187-97
AB  - Polycystic kidney diseases are characterized by the progressive expansion of
      multiple cystic lesions, which compromise the function of normal parenchyma.
      Throughout the course of these diseases, renal tubular function and structure are
      altered, changing the tubular microenvironment and ultimately causing the
      formation and progressive expansion of cystic lesions. Renal tubules are
      predisposed to cystogenesis when a germ line mutation is inherited in either the 
      human PKD1 or PKD2 genes in autosomal dominant polycystic kidney disease (ADPKD) 
      or when a homozygous mutation in Tg737 is inherited in the orpk mouse model of
      autosomal recessive polycystic kidney disease (ARPKD). Recent information
      strongly suggests that the protein products of these disease genes may form a
      macromolecular signaling structure, the polycystin complex, which regulates
      fundamental aspects of renal epithelial development and cell biology. Here, we
      re-examine the cellular pathophysiology of renal cyst formation and enlargement
      in the context of our current understanding of the molecular genetics of ADPKD
      and ARPKD.
FAU - Murcia, N S
AU  - Murcia NS
AD  - Department of Pediatrics, Rainbow Babies and Children's Hospital, Cleveland,
      Ohio, USA.
FAU - Sweeney, W E Jr
AU  - Sweeney WE Jr
FAU - Avner, E D
AU  - Avner ED
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Kidney Int
JT  - Kidney international
JID - 0323470
RN  - 0 (Growth Substances)
RN  - 0 (Proto-Oncogene Proteins)
RN  - EC 2.7.10.1 (ErbB Receptors)
SB  - IM
MH  - Animals
MH  - Apoptosis
MH  - Cell Division/genetics/physiology
MH  - Cyst Fluid/metabolism
MH  - ErbB Receptors/metabolism
MH  - Extracellular Matrix/metabolism/pathology
MH  - Growth Substances/biosynthesis
MH  - Humans
MH  - Polycystic Kidney, Autosomal Dominant/*genetics/metabolism/*physiopathology
MH  - Polycystic Kidney, Autosomal Recessive/*genetics/metabolism/*physiopathology
MH  - Proto-Oncogene Proteins/biosynthesis
MH  - Signal Transduction/physiology
RF  - 109
EDAT- 1999/04/14 02:03
MHDA- 2001/03/28 10:01
CRDT- 1999/04/14 02:03
PHST- 1999/04/14 02:03 [pubmed]
PHST- 2001/03/28 10:01 [medline]
PHST- 1999/04/14 02:03 [entrez]
AID - 10.1046/j.1523-1755.1999.00370.x [doi]
AID - S0085-2538(15)46070-3 [pii]
PST - ppublish
SO  - Kidney Int. 1999 Apr;55(4):1187-97. doi: 10.1046/j.1523-1755.1999.00370.x.