What question did this study set out to answer?

To determine whether the combined lipase deficiency (cld) mutation disrupts the folding and maturation of nascent lipoprotein lipase in the endoplasmic reticulum similarly to the lec23 mutation.

November 1, 1999Journal of Lipid ResearchOpen Access

cld and lec23 are disparate mutations that affect maturation of lipoprotein lipase in the endoplasmic reticulum

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Population

Cell lines homozygous for cld and lec23 mutations (Cld and Lec23) and livers from cld/cld mice

Design

Preclinical

Authors

VBVéronique Briquet-LaugierAgence Nationale de la Recherche OBOsnat Ben-ZeevWest Los Angeles College AWAnn L. WhiteUniversity of Georgia

Discussion

Member takes

Implication

Hypothesis-generating in cld/lec23 models; leaves open whether calnexin modulation affects LPL activity or hypertriglyceridemia in vivo.

Key Points

To determine whether the combined lipase deficiency (cld) mutation disrupts the folding and maturation of nascent lipoprotein lipase in the endoplasmic reticulum similarly to the lec23 mutation.
Analyzed lipoprotein lipase activity, aggregation, heparin affinity, and secretory trafficking in homozygous Cld and Lec23 mutant cell lines.
Profiled endoplasmic reticulum maturation factors and molecular chaperones in Cld cell cultures and liver tissue from cld/cld mice.
Both Cld and Lec23 cells produced predominantly inactive, heterogeneously aggregated lipoprotein lipase with impaired heparin affinity that was retained in the endoplasmic reticulum, allowing only a small properly folded fraction to reach secretion.
Calnexin levels were dramatically reduced in both cld/cld mouse liver tissues and Cld cells, implicating chaperone network disruption in the defective maturation of lipoprotein lipase.

Structured PICO

Population

Cell lines homozygous for cld and lec23 mutations (Cld and Lec23) and livers from cld/cld mice

Intervention

cld and lec23 mutations

Outcome

Lipoprotein lipase (LPL) activity, folding, assembly, and cellular localizationsurrogate

The cld and lec23 mutations disrupt lipoprotein lipase maturation by causing misfolding and retention in the endoplasmic reticulum, which may be related to reduced calnexin levels.

Cite This Study

Briquet-Laugier et al. (1999) studied this question.

synapsesocial.com/papers/6a702fd55d37378ac1dd4162 https://doi.org/10.1016/s0022-2275(20)32428-7

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cld and lec23 are disparate mutations that affect maturation of lipoprotein lipase in the endoplasmic reticulum

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

cld and lec23 are disparate mutations that affect maturation of lipoprotein lipase in the endoplasmic reticulum

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

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