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March 1, 1997Journal of Lipid ResearchOpen Access

Metabolism of apolipoprotein B-100 in a kindred with familial hypobetalipoproteinemia without a truncated form of apoB

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Key result

Familial hypobetalipoproteinemia without apoB truncations was associated with normal apoB-100 production rates but VLDL and LDL fractional catabolic rates 2 and 3 times higher than normal.

Population

4 affected members of a kindred with familial hypobetalipoproteinemia, 14 normal subjects, and 8 previously…

Comparison

8-h primed intravenous infusion of [13C]-leucine vs Normal subjects and FHBL subjects with apoB…

Design

Case-control

Authors

MLMarie LatourUniversity of New CaledoniaBPBruce W. PattersonWashington University in St. LouisJPJudit I. PulaiSaint George Hospital

Discussion

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Overview

May indicate non-apoB loci in FHBL; leaves open genetic mechanisms and clinical relevance pending confirmation.

Study Design

Type

Observational (n=26)

Structured PICO

P
Population
26 subjects, including 4 with FHBL without apoB truncations, 14 normal controls, and 8 with FHBL with apoB truncations, undergoing kinetic analysis.
E
Exposure
8-h primed intravenous infusion of [13C]-leucine
C
Comparator
Normal subjects and FHBL subjects with apoB truncations
O
Outcome
Kinetic parameters of apoB-100-containing lipoproteins (production rate and fractional catabolic rate)surrogate

Different genetic forms of familial hypobetalipoproteinemia are driven by distinct physiologic mechanisms, with non-truncation FHBL driven by increased catabolism rather than reduced production.

Cite This Study

Latour et al. (1997) conducted an observational in Familial hypobetalipoproteinemia (n=26). Familial hypobetalipoproteinemia without apoB truncations vs. Normal controls and FHBL with apoB truncations was evaluated on apoB-100 production rates and fractional catabolic rates. Familial hypobetalipoproteinemia without apoB truncations was associated with normal apoB-100 production rates but VLDL and LDL fractional catabolic rates 2 and 3 times higher than normal.

synapsesocial.com/papers/6a6ba712547974b2dbf45bcbhttps://doi.org/10.1016/s0022-2275(20)37267-9
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1ApoB-75, a truncation of apolipoprotein B associated with familial hypobetalipoproteinemia: genetic and kinetic studies.1992 · 95 citations
  2. 2Apolipoprotein B-38.9 does not associate with apo[a] and forms two distinct HDL density particle populations that are larger than HDL.1994 · 22 citations
  3. 3Organ Loci of Catabolism of Short Truncations of ApoB1997 · 25 citations
  4. 4A quick and large-scale density gradient subfractionation method for low density lipoproteins.1982 · 63 citations
  5. 5Tracer-to-tracee ratio for analysis of stable isotope tracer data: link with radioactive kinetic formalism1992 · 177 citations