PMID- 1639819
OWN - NLM
STAT- MEDLINE
DCOM- 19920828
LR  - 20210210
IS  - 0021-9258 (Print)
IS  - 0021-9258 (Linking)
VI  - 267
IP  - 22
DP  - 1992 Aug 5
TI  - Deficiencies in sex-regulated expression and levels of two hepatic sterol carrier 
      proteins in a murine model of Niemann-Pick type C disease.
PG  - 15902-8
AB  - Hepatic sterol carrier protein-2 (SCP2) and sterol carrier protein-X (SCPx) 
      levels in normal and in mutant Niemann-Pick Type C mice were determined by 
      immunoblotting with antiserum against rat SCP2. A 14-kDa protein (SCP2) was 
      detected in the cytosol fraction and a 58-kDa protein (SCPx) was found in both 
      cytosolic and organellar fractions. Expression of hepatic SCPx protein was 
      developmentally regulated in a sex-specific pattern. The amounts of 
      organelle-associated SCPx increased 4-fold during sexual development of normal 
      males but decreased dramatically during development of normal females. Levels of 
      hepatic SCP2 increased much less dramatically during sexual maturation of normal 
      males and females. Adult Niemann-Pick Type C mice were deficient in both hepatic 
      SCPx and SCP2. The deficit in SCPx in affected males reflected a failure to 
      increase hepatic SCPx levels during sexual maturation. In affected males SCPx 
      remained at levels found in immature mice. Affected male and female mice were 
      also unable to maintain levels of hepatic SCP2. The level of SCP2 was near normal 
      in affected immature males and subnormal in affected immature females. During 
      sexual maturation hepatic SCP2 declined in affected animals.
FAU - Roff, C F
AU  - Roff CF
AD  - Developmental and Metabolic Neurology Branch, National Institute of Neurological 
      Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892.
FAU - Pastuszyn, A
AU  - Pastuszyn A
FAU - Strauss, J F 3rd
AU  - Strauss JF 3rd
FAU - Billheimer, J T
AU  - Billheimer JT
FAU - Vanier, M T
AU  - Vanier MT
FAU - Brady, R O
AU  - Brady RO
FAU - Scallen, T J
AU  - Scallen TJ
FAU - Pentchev, P G
AU  - Pentchev PG
LA  - eng
GR  - DK-10628/DK/NIDDK NIH HHS/United States
GR  - HD-06274/HD/NICHD NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - J Biol Chem
JT  - The Journal of biological chemistry
JID - 2985121R
RN  - 0 (Carrier Proteins)
RN  - 0 (Plant Proteins)
RN  - 0 (Sterols)
RN  - 0 (sterol carrier proteins)
RN  - EC 1.11.1.6 (Catalase)
SB  - IM
MH  - Aging
MH  - Animals
MH  - Carrier Proteins/genetics/*metabolism
MH  - Catalase/metabolism
MH  - Cytosol/metabolism
MH  - Female
MH  - Gene Expression Regulation
MH  - Liver/growth & development/*metabolism
MH  - Male
MH  - Mice
MH  - Mice, Inbred BALB C
MH  - Molecular Weight
MH  - Niemann-Pick Diseases/genetics/*metabolism
MH  - Organelles/metabolism
MH  - *Plant Proteins
MH  - Sex Characteristics
MH  - Sterols/*metabolism
EDAT- 1992/08/05 00:00
MHDA- 1992/08/05 00:01
CRDT- 1992/08/05 00:00
PHST- 1992/08/05 00:00 [pubmed]
PHST- 1992/08/05 00:01 [medline]
PHST- 1992/08/05 00:00 [entrez]
AID - S0021-9258(19)49619-1 [pii]
PST - ppublish
SO  - J Biol Chem. 1992 Aug 5;267(22):15902-8.