PMID- 7977782
OWN - NLM
STAT- MEDLINE
DCOM- 19941208
LR  - 20171213
IS  - 0002-9513 (Print)
IS  - 0002-9513 (Linking)
VI  - 267
IP  - 5 Pt 2
DP  - 1994 Nov
TI  - Coordinate regulation of 11 beta-HSD and Ke 6 genes in cpk mouse: implications 
      for steroid metabolic defect in PKD.
PG  - F791-7
AB  - Polycystic kidney disease (PKD) is characterized by multiple renal cysts, which 
      ultimately result in renal failure. We have reported the identification of a new 
      gene, Ke 6, within the major histocompatibility complex, which is downregulated 
      in two different mouse models of heritable PKD (N. Aziz, M. Maxwell, B. 
      St.-Jacques, and B.M. Brenner. Mol. Cell. Biol. 13: 1847-1853, 1993). The Ke 6 
      gene gives rise to two transcripts, Ke 6a and Ke 6b. Ke 6a protein has 
      significant homology to several mammalian and bacterial steroid dehydrogenases. 
      The homology of Ke 6a protein to specific functional domains of the human and rat 
      11 beta-hydroxysteroid dehydrogenase enzyme (11 beta-HSD), which inactivates 
      glucocorticoids, is substantial. We report here that the Ke 6 gene and the 11 
      beta-HSD gene are regulated in the same aberrant pattern in the cpk mouse. The 
      strong evidence for a critical role of steroids in cystogenesis leads us to 
      propose that a possible elevation of intrahepatic and intrarenal steroid levels 
      occurs in the cpk mouse as a result of repression of steroid metabolic enzymes, 
      which ultimately leads to development of cysts.
FAU - Aziz, N
AU  - Aziz N
AD  - Department of Medicine, Children's Hospital, Boston, Massachusetts.
FAU - Maxwell, M M
AU  - Maxwell MM
FAU - Brenner, B M
AU  - Brenner BM
LA  - eng
GR  - DK-35930/DK/NIDDK NIH HHS/United States
GR  - DK-48098/DK/NIDDK NIH HHS/United States
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Am J Physiol
JT  - The American journal of physiology
JID - 0370511
RN  - 0 (Glucocorticoids)
RN  - 0 (H2-Ke6 protein, mouse)
RN  - 0 (Histocompatibility Antigens)
RN  - 0 (RNA, Messenger)
RN  - 0 (Steroids)
RN  - EC 1.- (Oxidoreductases)
RN  - EC 1.1.- (Hydroxysteroid Dehydrogenases)
RN  - EC 1.1.1.- (Hydroxyprostaglandin Dehydrogenases)
RN  - EC 1.1.1.141 (15-hydroxyprostaglandin dehydrogenase)
RN  - EC 1.1.1.146 (11-beta-Hydroxysteroid Dehydrogenases)
SB  - IM
GS  - Ke 6
MH  - 11-beta-Hydroxysteroid Dehydrogenases
MH  - Amino Acid Sequence
MH  - Animals
MH  - Base Sequence
MH  - Blotting, Northern
MH  - Consensus Sequence
MH  - Conserved Sequence
MH  - *Gene Expression Regulation
MH  - Gene Expression Regulation, Enzymologic
MH  - Genetic Carrier Screening
MH  - Glucocorticoids/metabolism
MH  - Histocompatibility Antigens/*biosynthesis/genetics
MH  - Humans
MH  - Hydroxyprostaglandin Dehydrogenases/biosynthesis/genetics
MH  - Hydroxysteroid Dehydrogenases/*biosynthesis/genetics
MH  - Kidney/enzymology/immunology
MH  - Liver/enzymology/immunology
MH  - *Major Histocompatibility Complex
MH  - Mammals
MH  - Mice
MH  - Mice, Inbred DBA
MH  - Mice, Mutant Strains
MH  - Molecular Sequence Data
MH  - *Oxidoreductases
MH  - Polycystic Kidney Diseases/*genetics/immunology/metabolism
MH  - RNA, Messenger/isolation & purification/metabolism
MH  - Rats
MH  - Regulatory Sequences, Nucleic Acid
MH  - Sequence Homology, Amino Acid
MH  - Steroids/*metabolism
MH  - Transcription, Genetic
EDAT- 1994/11/01 00:00
MHDA- 1994/11/01 00:01
CRDT- 1994/11/01 00:00
PHST- 1994/11/01 00:00 [pubmed]
PHST- 1994/11/01 00:01 [medline]
PHST- 1994/11/01 00:00 [entrez]
AID - 10.1152/ajprenal.1994.267.5.F791 [doi]
PST - ppublish
SO  - Am J Physiol. 1994 Nov;267(5 Pt 2):F791-7. doi: 10.1152/ajprenal.1994.267.5.F791.