mmpc-logo mmpc-logo
twitter-logo    bluesky-logo
| Create Account | login
Publication
Adipocyte-Specific Deletion of Lamin A/C Largely Models Human Familial Partial
Lipodystrophy Type 2.
Authors Corsa CAS, Walsh CM, Bagchi DP, Foss Freitas MC, Li Z, Hardij J, Granger K, Mori
H, Schill RL, Lewis KT, Maung JN, Azaria RD, Rothberg AE, Oral EA, MacDougald OA
Submitted By Submitted Externally on 12/3/2021
Status Published
Journal Diabetes
Year 2021
Date Published 9/1/2021
Volume : Pages 70 : 1970 - 1984
PubMed Reference 34088712
Abstract Mechanisms by which autosomal recessive mutations in Lmna cause familial partial
lipodystrophy type 2 (FPLD2) are poorly understood. To investigate the function
of lamin A/C in adipose tissue, we created mice with an adipocyte-specific loss
of Lmna (LmnaADKO). Although LmnaADKO mice develop and maintain adipose tissues
in early postnatal life, they show a striking and progressive loss of white and
brown adipose tissues as they approach sexual maturity. LmnaADKO mice exhibit
surprisingly mild metabolic dysfunction on a chow diet, but on a high-fat diet
they share many characteristics of FPLD2 including hyperglycemia, hepatic
steatosis, hyperinsulinemia, and almost undetectable circulating adiponectin and
leptin. Whereas LmnaADKO mice have reduced regulated and constitutive bone
marrow adipose tissue with a concomitant increase in cortical bone, FPLD2
patients have reduced bone mass and bone mineral density compared with controls.
In cell culture models of Lmna deficiency, mesenchymal precursors undergo
adipogenesis without impairment, whereas fully differentiated adipocytes have
increased lipolytic responses to adrenergic stimuli. LmnaADKO mice faithfully
reproduce many characteristics of FPLD2 and thus provide a unique animal model
to investigate mechanisms underlying Lmna-dependent loss of adipose tissues.




Menu

Home
Contact
About MMPC
Animal Husbandry
Tests Data
Search Data
Analysis
Clients
MMPC Centers

Newsletter

Interested in receiving MMPC News?
twitter-logo Mouse Phenotyping
@NationalMMPC



2017 National MMPC. All Rights Reserved.