Linked Life Data

3128686

Source:http://linkedlifedata.com/resource/pubmed/id/3128686

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1988-5-13
pubmed:abstractText
Acidic alpha-mannosidase deficiency has been identified in a family of Blue Persian cats. Characterization of the residual activity revealed that the Km for the substrate, 4-methylumbelliferyl-alpha-D-mannoside, increased approximately three-fold with a severe deficiency in Vmax (1-2%) in homogenates of liver and brain of affected cats compared with controls. The residual activity at pH 4.0 in liver homogenates from affected cats is very thermolabile at 51 degrees C while the control activity is stable at this temperature for 1 h. Subcellular fractionation of liver was performed from a control and diseased cat in order to compare the properties of the different alpha-mannosidases localized in these fractions. The residual activity present in the lysosomal fraction from diseased cat liver showed altered pH optimum, two-fold increase in Km with a severely reduced Vmax and increased thermolability compared with the activity in the lysosomal fraction from control liver. The thermal inactivation pattern and Km of the residual activity in the lysosomal fraction is different from the non-lysosomal alpha-mannosidase in the liver of the affected cat. This suggests that the residual activity in the lysosomal fraction of the liver from the affected cat is not due to contamination of non-lysosomal alpha-mannosidase in this fraction. Whether this residual activity represents the properties of the mutant enzyme or yet another minor normal component of lysosomes different from the major inactive mutant or absent lysosomal enzyme remains to be elucidated.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0141-8955
pubmed:author
pubmed:issnType
Print
pubmed:volume
11
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
3-16
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1988
pubmed:articleTitle
Characterization of alpha-mannosidase in feline mannosidosis.
pubmed:affiliation
Department of Biochemistry, Eunice Kennedy Shriver Center for Mental Retardation, Inc., Waltham, MA 02254.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Research Support, Non-U.S. Gov't