Tissue and cellular specific expression of murine lysosomal acid lipase mRNA and protein

Hong Du, David P. Witte, Gregory A. Grabowski

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

Lysosomal acid lipase (LAL) is essential to the intracellular control of cholesterol and triglyceride catabolism via the low density lipoprotein (LDL), delivery of these neutral lipids to the lysosome. Deficiency of LAL in humans leads to Wolman disease and cholesteryl ester storage disease that result, respectively, in the intralysosomal storage of both neutral lipids or only cholesteryl esters. The mouse and human LAL cDNAs were cloned. The deduced amino acid sequences from the mouse and human LAL had high similarity (95%) and identity (75%) including conservation of the active center motifs (G-X-S-X-G) and five potential N-glycosylation consensus sequences. Tissue specific expression of LAL mRNA and protein in mouse tissues was evaluated by in situ hybridization and immunofluorescence staining, respectively. The LAL mRNA was expressed at low levels in most tissues. High level expression was found in hepatocytes and splenic and thymic cells. Very high level expression was observed in cells of the small intestinal villi, the zona fasciculata and reticularis of the adrenal cortex, pancreatic acini, and renal tubular epithelium. Significant levels of expression were detected in epithelial cells of choroid plexus in developing mouse embryo by day 12, in liver and lung by day 14, and in small intestine and kidney by day 16. Similar distribution of LAL protein was observed by immunofluorescence stain. Our results show that the expression of LAL is regulated in a tissue- and cell- specific manner that corresponds to the pathologic involvement in Wolman disease.

Original languageEnglish (US)
Pages (from-to)937-949
Number of pages13
JournalJournal of Lipid Research
Volume37
Issue number5
StatePublished - May 1 1996
Externally publishedYes

Fingerprint

Sterol Esterase
Tissue
Wolman Disease
Messenger RNA
Proteins
Cholesterol Esters
Fluorescent Antibody Technique
Cholesterol Ester Storage Disease
Zona Reticularis
Zona Fasciculata
Kidney
Lipids
Choroid Plexus
Adrenal Cortex
Glycosylation
Consensus Sequence
Lysosomes
LDL Lipoproteins
Small Intestine
In Situ Hybridization

Keywords

  • cholesteryl esterase
  • gene expression
  • immunofluorescence
  • in situ hybridization
  • triglyceridase

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology
  • Cell Biology

Cite this

Tissue and cellular specific expression of murine lysosomal acid lipase mRNA and protein. / Du, Hong; Witte, David P.; Grabowski, Gregory A.

In: Journal of Lipid Research, Vol. 37, No. 5, 01.05.1996, p. 937-949.

Research output: Contribution to journalArticle

Du, Hong ; Witte, David P. ; Grabowski, Gregory A. / Tissue and cellular specific expression of murine lysosomal acid lipase mRNA and protein. In: Journal of Lipid Research. 1996 ; Vol. 37, No. 5. pp. 937-949.
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