Evidence mapPaperPMID 40367289Full record

ArticleThe Journal of clinical endocrinology and metabolism2025

Novel and Ultrarare Heterozygous Missense LMNA Variants Causing Familial Partial Lipodystrophy.

Anum, Xilong Li, Abhimanyu Garg

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Article in The Journal of clinical endocrinology and metabolism, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

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5citing papers in PubMed
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5 citing papers in PubMed.

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5 · Who and what money

Authors and funding

3 authors.

AnumThe Section of Nutrition and Metabolic Diseases, Division of Endocrinology, Department of Internal Medicine and the Center for Human Nutrition, UT Southwestern Medical Center, Dallas, TX 75390-8537, USA.
Xilong LiPeter O'Donnell Jr. School of Public Health, UT Southwestern Medical Center, Dallas, TX 75390-9066, USA.
Abhimanyu GargThe Section of Nutrition and Metabolic Diseases, Division of Endocrinology, Department of Internal Medicine and the Center for Human Nutrition, UT Southwestern Medical Center, Dallas, TX 75390-8537, USA.ORCID 0000-0001-7209-6986

Funding

NCATS NIH HHSNIH HHS R01-DK101941NIH HHS UL1TR001105
6 · The paper itself

Abstract

contextFamilial partial lipodystrophy, type 2 (FPLD2) or the Dunnigan variety, is a rare, autosomal dominant disorder characterized by selective loss of subcutaneous fat from the extremities and is caused by over 50 heterozygous missense LMNA variants. However, some patients with FPLD2 do not harbor the known pathogenic LMNA variants and there are only limited genotype-phenotype segregation data for a few other variants.

objectiveTo report the genotype-phenotype relationships in 4 families with ultrarare and novel LMNA variants causing FPLD2.

methodsClinical, anthropometric, and laboratory data of affected and unaffected subjects from 4 families with female probands presenting with FPLD2 phenotype were collected retrospectively. The main parameters were clinical phenotype, skinfold thickness, regional body fat by dual-energy X-ray absorptiometry (DXA), metabolic variables, and prevalence of diabetes mellitus and hypertriglyceridemia.

resultsWe found 2 ultrarare (p.N466D, and p.K515E) and 2 novel (p.R582S, and p.L241P) LMNA heterozygous variants in 4 unrelated FPLD2 families. All adult affected females had thigh skinfold thickness below the 10th percentile of normal and lower extremity fat below the 1st percentile of normal suggesting "typical" FPLD2. None of our patients had any cardiomyopathy, muscular dystrophy, neuropathy, or any progeroid features.

conclusionOur data provide further supporting evidence for the pathogenicity of 2 previously reported ultrarare heterozygous LMNA variants, p.N466D and p.K515E. We also report 2 novel variants, p.R582S and p.L241P, in patients with FPLD2. Thus, our study broadens the spectrum of pathogenic/likely pathogenic LMNA variants in FPLD2.

Indexed as

Lamin Type ALipodystrophy, Familial PartialMutation, MissenseAdultFemaleGenetic Association StudiesHeterozygoteHumansMaleMiddle AgedPedigreePhenotypeRetrospective StudiesYoung AdultLamin Type ALMNA protein, humanDunnigan varietyfamilial partial lipodystrophyFPLD2lamin A/CLMNA variant

Identifiers

PMID40367289
PMCPMC12712948

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.