Journal of Inherited Metabolic Disease

, Volume 32, Issue 6, pp 684–698

Mitochondria and diabetes mellitus: untangling a conflictive relationship?

  • M. Schiff
  • S. Loublier
  • A. Coulibaly
  • P. Bénit
  • H. Ogier de Baulny
  • P. Rustin
REVIEW

DOI: 10.1007/s10545-009-1263-0

Cite this article as:
Schiff, M., Loublier, S., Coulibaly, A. et al. J Inherit Metab Dis (2009) 32: 684. doi:10.1007/s10545-009-1263-0
  • 340 Downloads

Summary

Diabetes mellitus is occasionally observed in patients with skeletal muscle respiratory chain deficiency, suggesting that skeletal muscle mitochondrial dysfunction might play a pathogenic role in type 2 diabetes (T2D). In support of this hypothesis, decreased muscle mitochondrial activity has been reported in T2D patients and in mouse models of diabetes. However, recent work by several groups suggests that decreased muscle mitochondrial function may be a consequence rather than a cause of diabetes, since decreased mitochondrial function in mice affords protection from diabetes and obesity. We review the data on this controversial but important issue of potential links between mitochondrial dysfunction and diabetes.

Abbreviations

AIF

apoptosis-inducing factor

HNF-1α

hepatocyte nuclear factor-1α

IMCL

intramyocellular lipid

MELAS

mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes

MIDD

maternally inherited diabetes and deafness syndrome

MODY

maturity-onset diabetes of the young

PGC-1α

peroxisome proliferator-activated receptor γ co-activator 1α

PPAR

peroxisome proliferator-activated receptor

T2D

type 2 diabetes

TRMA

thiamine-responsive megaloblastic anaemia

UCP2

uncoupling protein 2

Copyright information

© SSIEM and Springer 2009

Authors and Affiliations

  • M. Schiff
    • 1
    • 2
    • 3
  • S. Loublier
    • 1
    • 2
  • A. Coulibaly
    • 1
    • 2
  • P. Bénit
    • 1
    • 2
  • H. Ogier de Baulny
    • 3
  • P. Rustin
    • 1
    • 2
    • 4
  1. 1.Hôpital Robert DebréParisFrance
  2. 2.Université Paris 7, Faculté de médecine Denis DiderotParisFrance
  3. 3.Centre de référence Maladies MétaboliquesHôpital Robert DebréParisFrance
  4. 4.INSERM U676, Bâtiment Ecran, Hôpital Robert DebréParisFrance

Personalised recommendations