Mitochondrial phospholipid metabolism in health and disease.
Barth syndrome
Cardiolipin
Membranes
Mitochondria
Phosphatidylethanolamine
Phospholipids
Journal
Journal of cell science
ISSN: 1477-9137
Titre abrégé: J Cell Sci
Pays: England
ID NLM: 0052457
Informations de publication
Date de publication:
01 09 2023
01 09 2023
Historique:
pmc-release:
01
09
2024
medline:
8
9
2023
pubmed:
1
9
2023
entrez:
1
9
2023
Statut:
ppublish
Résumé
Studies of rare human genetic disorders of mitochondrial phospholipid metabolism have highlighted the crucial role that membrane phospholipids play in mitochondrial bioenergetics and human health. The phospholipid composition of mitochondrial membranes is highly conserved from yeast to humans, with each class of phospholipid performing a specific function in the assembly and activity of various mitochondrial membrane proteins, including the oxidative phosphorylation complexes. Recent studies have uncovered novel roles of cardiolipin and phosphatidylethanolamine, two crucial mitochondrial phospholipids, in organismal physiology. Studies on inter-organellar and intramitochondrial phospholipid transport have significantly advanced our understanding of the mechanisms that maintain mitochondrial phospholipid homeostasis. Here, we discuss these recent advances in the function and transport of mitochondrial phospholipids while describing their biochemical and biophysical properties and biosynthetic pathways. Additionally, we highlight the roles of mitochondrial phospholipids in human health by describing the various genetic diseases caused by disruptions in their biosynthesis and discuss advances in therapeutic strategies for Barth syndrome, the best-studied disorder of mitochondrial phospholipid metabolism.
Identifiants
pubmed: 37655851
pii: 326600
doi: 10.1242/jcs.260857
pmc: PMC10482392
pii:
doi:
Substances chimiques
Phospholipids
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : NIGMS NIH HHS
ID : R01 GM111672
Pays : United States
Organisme : NIGMS NIH HHS
ID : R01 GM143630
Pays : United States
Informations de copyright
© 2023. Published by The Company of Biologists Ltd.
Déclaration de conflit d'intérêts
Competing interests The authors declare no competing or financial interests.
Références
Nat Commun. 2019 Mar 29;10(1):1432
pubmed: 30926815
Proc Natl Acad Sci U S A. 2020 Feb 25;117(8):4061-4070
pubmed: 32041880
Circulation. 2021 Nov 23;144(21):1694-1713
pubmed: 34648376
Biol Chem. 2020 May 26;401(6-7):811-820
pubmed: 32049644
Front Genet. 2015 Feb 03;6:3
pubmed: 25691889
Traffic. 2014 Sep;15(9):933-45
pubmed: 24954234
Traffic. 2012 Jun;13(6):880-90
pubmed: 22409400
EMBO J. 2010 Jun 16;29(12):1976-87
pubmed: 20485265
Am J Med Genet A. 2013 Sep;161A(9):2204-15
pubmed: 23918762
Biochim Biophys Acta Mol Cell Biol Lipids. 2020 Jun;1865(6):158655
pubmed: 32058032
Genet Med. 2021 Mar;23(3):471-478
pubmed: 33077895
J Biol Chem. 2019 Jul 26;294(30):11568-11578
pubmed: 31186346
Ann Neurol. 2017 Dec;82(6):1004-1015
pubmed: 29205472
Sci Rep. 2020 Feb 18;10(1):2845
pubmed: 32071354
J Biol Chem. 2009 Apr 24;284(17):11572-8
pubmed: 19244244
J Biol Chem. 2016 Sep 2;291(36):18718-29
pubmed: 27402832
J Cell Biol. 2005 Jun 6;169(5):801-11
pubmed: 15939762
Biochim Biophys Acta Mol Cell Biol Lipids. 2018 Mar;1863(3):284-298
pubmed: 29253589
Mol Biol Cell. 2016 Jul 15;27(14):2161-71
pubmed: 27226479
Science. 2012 Nov 9;338(6108):815-8
pubmed: 23042293
Mol Cell Biochem. 2021 Mar;476(3):1605-1629
pubmed: 33415565
Nat Cell Biol. 2022 Jun;24(6):996-1004
pubmed: 35654841
J Biol Chem. 1998 Apr 17;273(16):9829-36
pubmed: 9545322
Cell Rep. 2020 Mar 24;30(12):4281-4291.e4
pubmed: 32209484
J Cell Biol. 2022 Dec 5;221(12):
pubmed: 36282247
Biochemistry. 2008 Apr 15;47(15):4518-29
pubmed: 18358005
EMBO J. 2018 Feb 15;37(4):
pubmed: 29301859
Biochim Biophys Acta. 2004 Nov 3;1666(1-2):62-87
pubmed: 15519309
J Biol Chem. 2021 Sep;297(3):101005
pubmed: 34314685
Biochem Biophys Res Commun. 2015 Aug 21;464(2):580-5
pubmed: 26164234
FEBS Lett. 2006 May 29;580(13):3059-64
pubmed: 16678169
Mol Biol Cell. 2016 Aug 1;27(15):2435-49
pubmed: 27280386
J Biol Chem. 2004 Oct 8;279(41):42612-8
pubmed: 15292198
Sci Adv. 2023 Feb 24;9(8):eade7864
pubmed: 36827367
J Cell Biol. 2016 Jun 6;213(5):525-34
pubmed: 27241913
Dev Cell. 2018 Jan 22;44(2):261-270.e6
pubmed: 29290583
Life Sci Alliance. 2019 Mar 11;2(2):
pubmed: 30858161
Pediatr Neurol. 2020 Sep;110:25-29
pubmed: 32684373
Cell Rep. 2022 Sep 20;40(12):111364
pubmed: 36130504
J Neurol Sci. 1983 Dec;62(1-3):327-55
pubmed: 6142097
Sci Adv. 2020 Aug 28;6(35):eabb0780
pubmed: 32923632
J Biol Chem. 2020 Aug 14;295(33):11928-11937
pubmed: 32636300
Pediatr Neurol. 2008 Aug;39(2):113-5
pubmed: 18639755
Sci Rep. 2017 Aug 18;7(1):8793
pubmed: 28821867
J Cell Biol. 1994 Sep;126(6):1375-91
pubmed: 8089172
Biochim Biophys Acta Mol Basis Dis. 2022 Jan 1;1868(1):166298
pubmed: 34751152
Nature. 2013 Sep 12;501(7466):257-61
pubmed: 23934110
J Biol Chem. 2012 May 4;287(19):15205-18
pubmed: 22403410
J Biol Chem. 1992 Aug 25;267(24):16790-5
pubmed: 1512221
Mol Cell. 2017 Aug 3;67(3):471-483.e7
pubmed: 28712724
Cell Biochem Biophys. 2003;38(2):161-90
pubmed: 12777713
Nat Genet. 2012 Jun 10;44(7):797-802
pubmed: 22683713
Hum Mol Genet. 2023 Jun 5;32(12):2055-2067
pubmed: 36917259
J Intern Med. 2020 Jun;287(6):645-664
pubmed: 32012363
Biochim Biophys Acta. 2015 Dec;1851(12):1566-76
pubmed: 26417903
Hum Gene Ther. 2019 Feb;30(2):139-154
pubmed: 30070157
Cell Metab. 2018 Jul 03;28(1):159-174.e11
pubmed: 29861389
Proc Natl Acad Sci U S A. 2021 Jul 20;118(29):
pubmed: 34261790
Front Mol Biosci. 2022 May 27;9:915301
pubmed: 35693555
Autophagy. 2022 Dec;18(12):2985-3003
pubmed: 35414338
Biophys J. 2007 Dec 1;93(11):3884-99
pubmed: 17704167
J Cell Biol. 2018 Mar 5;217(3):959-974
pubmed: 29279306
Proc Natl Acad Sci U S A. 2015 Jun 23;112(25):E3179-88
pubmed: 26056272
Lipids. 1994 Jul;29(7):475-80
pubmed: 7968268
J Inherit Metab Dis. 2022 Jan;45(1):60-71
pubmed: 34626131
Nat Commun. 2022 Jun 28;13(1):3702
pubmed: 35764626
EMBO J. 2010 Sep 1;29(17):2888-98
pubmed: 20657548
Mol Cell. 2023 Mar 16;83(6):890-910
pubmed: 36931257
Ophthalmic Paediatr Genet. 1986 Dec;7(3):151-8
pubmed: 3561949
Biochim Biophys Acta. 1985 Jun 12;822(1):1-42
pubmed: 2408671
J Cell Biol. 2018 Oct 1;217(10):3625-3639
pubmed: 30093493
J Biol Chem. 2013 Jun 7;288(23):16451-16459
pubmed: 23625917
Mol Cell Biol. 2011 Sep;31(18):3845-56
pubmed: 21746876
J Biol Chem. 2003 Dec 19;278(51):51380-5
pubmed: 14551214
J Biol Chem. 1993 Oct 5;268(28):21416-24
pubmed: 8407984
J Cell Biol. 2000 Oct 30;151(3):551-62
pubmed: 11062257
J Biol Chem. 2010 Mar 5;285(10):7358-65
pubmed: 20042613
J Bacteriol. 1999 Mar;181(5):1458-63
pubmed: 10049376
Orphanet J Rare Dis. 2014 Aug 20;9:119
pubmed: 25208612
JMIR Res Protoc. 2021 May 31;10(5):e22533
pubmed: 34057417
Cell Discov. 2020 Sep 29;6:67
pubmed: 33083003
J Cell Biol. 2016 Jul 4;214(1):77-88
pubmed: 27354379
FEBS Lett. 2018 Apr;592(8):1273-1290
pubmed: 29067684
J Cell Biol. 2015 Sep 14;210(6):883-90
pubmed: 26370498
Science. 2018 Dec 7;362(6419):1186-1189
pubmed: 30361388
Proc Natl Acad Sci U S A. 2020 Jul 14;117(28):16383-16390
pubmed: 32601238
IUBMB Life. 2019 Jul;71(7):791-801
pubmed: 30746873
Front Cell Dev Biol. 2021 Nov 10;9:774989
pubmed: 34858991
Sci Adv. 2017 Sep 01;3(9):e1700532
pubmed: 28879236
Orphanet J Rare Dis. 2013 May 08;8:70
pubmed: 23656970
J Biol Chem. 2006 Dec 22;281(51):39217-24
pubmed: 17082194
Am J Hum Genet. 2012 Feb 10;90(2):314-20
pubmed: 22284826
J Cell Biol. 2020 Aug 3;219(8):
pubmed: 32614384
Biochim Biophys Acta. 1997 Apr 3;1325(1):108-16
pubmed: 9106488
J Cell Biol. 2020 Nov 2;219(11):
pubmed: 32926082
Mol Cell. 2016 Sep 15;63(6):1034-43
pubmed: 27635761
Prog Lipid Res. 2013 Oct;52(4):590-614
pubmed: 24007978
Proc Natl Acad Sci U S A. 2019 Jun 4;116(23):11235-11240
pubmed: 31110016
Biochim Biophys Acta. 2004 Nov 3;1666(1-2):275-88
pubmed: 15519321
Cell Metab. 2011 Jun 8;13(6):690-700
pubmed: 21641550
Sci Transl Med. 2012 Jan 25;4(118):118ra10
pubmed: 22277967
Biochimie. 2017 Nov;142:102-111
pubmed: 28842204
Mol Microbiol. 1997 Nov;26(3):481-91
pubmed: 9402019
Antioxidants (Basel). 2022 Nov 23;11(12):
pubmed: 36552523
Curr Biol. 2009 Dec 29;19(24):2133-9
pubmed: 19962311
J Inherit Metab Dis. 2022 Jan;45(1):29-37
pubmed: 34382226
Nat Genet. 1996 Apr;12(4):385-9
pubmed: 8630491
Dev Cell. 2014 Jul 14;30(1):86-94
pubmed: 25026035
Hum Mol Genet. 2022 Feb 3;31(3):376-385
pubmed: 34494107
EMBO Rep. 2016 Jun;17(6):800-10
pubmed: 27113756
Proc Natl Acad Sci U S A. 2017 Nov 7;114(45):E9502-E9511
pubmed: 29078410
J Biol Chem. 2021 Jan-Jun;296:100539
pubmed: 33722607
Science. 2009 Jul 24;325(5939):477-81
pubmed: 19556461
Genet Med. 2019 Dec;21(12):2734-2743
pubmed: 31263216
Cell Metab. 2013 Aug 6;18(2):287-95
pubmed: 23931759
Protein Cell. 2021 Mar;12(3):220-229
pubmed: 32862359
J Biol Chem. 2012 May 18;287(21):17589-17597
pubmed: 22433850
Mol Genet Metab. 2006 May;88(1):47-52
pubmed: 16527507
Dev Cell. 2014 Jul 14;30(1):95-102
pubmed: 25026036
Trends Cell Biol. 2018 Jan;28(1):67-76
pubmed: 28911913
Front Genet. 2016 Jan 20;6:359
pubmed: 26834781
Curr Biol. 2017 Jul 10;27(13):R629-R631
pubmed: 28697355
J Biol Chem. 1995 May 12;270(19):11190-8
pubmed: 7744750
J Biol Chem. 2004 Jul 23;279(30):31727-34
pubmed: 15152008
J Mol Cell Cardiol. 2020 Jul;144:24-34
pubmed: 32418915
Eur J Biochem. 1999 Sep;264(2):545-53
pubmed: 10491102
J Biol Chem. 2005 Aug 19;280(33):29403-8
pubmed: 15972817
Cell Death Differ. 2016 Jul;23(7):1140-51
pubmed: 26742431
J Biol Chem. 2013 Feb 8;288(6):4158-73
pubmed: 23250747
iScience. 2021 Jul 24;24(8):102899
pubmed: 34401677
Sci Rep. 2016 Jul 29;6:30777
pubmed: 27469264
Biophys J. 2018 Dec 18;115(12):2386-2394
pubmed: 30503532
J Biol Chem. 2009 Oct 30;284(44):30360-71
pubmed: 19737925
Hum Mol Genet. 2022 Oct 28;31(21):3597-3612
pubmed: 35147173
Autophagy. 2022 Sep;18(9):2134-2149
pubmed: 34985382
Sci Adv. 2019 Sep 11;5(9):eaax8352
pubmed: 31535029
J Biol Chem. 2007 Sep 28;282(39):28344-28352
pubmed: 17652094
J Pediatr. 1975 Jun;86(6):873-80
pubmed: 1168700
EMBO J. 2021 Dec 1;40(23):e108428
pubmed: 34661298
J Lipid Res. 2022 Jun;63(6):100227
pubmed: 35569528
J Biol Chem. 2005 Dec 2;280(48):40032-40
pubmed: 16192276
Commun Biol. 2019 Jun 20;2:225
pubmed: 31240263
Trends Cell Biol. 2002 Apr;12(4):178-84
pubmed: 11978537
Nat Cell Biol. 2017 Jul;19(7):856-863
pubmed: 28628083
Nat Cell Biol. 2013 Oct;15(10):1197-1205
pubmed: 24036476
Nucleic Acids Res. 2007 Jan;35(Database issue):D527-32
pubmed: 17098933
Mol Cell. 2017 Nov 16;68(4):786-796.e6
pubmed: 29149599
Mol Genet Metab. 2012 Nov;107(3):428-32
pubmed: 23031367
Yeast. 1995 May;11(6):493-536
pubmed: 7645343
Nature. 2014 Jun 5;510(7503):48-57
pubmed: 24899304
J Cell Biol. 2009 Jun 15;185(6):1029-45
pubmed: 19506038
Biochim Biophys Acta Mol Cell Biol Lipids. 2018 Aug;1863(8):857-865
pubmed: 29694924
Nat Cell Biol. 2006 Nov;8(11):1255-62
pubmed: 17028579
J Biochem. 2019 Feb 1;165(2):115-123
pubmed: 30371789
Langmuir. 2017 Jul 11;33(27):6937-6946
pubmed: 28628337
Hum Mutat. 2019 Mar;40(3):299-309
pubmed: 30488656
Science. 2015 Jul 24;349(6246):432-6
pubmed: 26206936
J Biomed Res. 2017 Nov 01;32(2):107-112
pubmed: 29336355
J Inherit Metab Dis. 2015 Jan;38(1):99-110
pubmed: 25178427
Br J Pharmacol. 2014 Apr;171(8):2029-50
pubmed: 24117165
EMBO Rep. 2021 Jun 4;22(6):e51323
pubmed: 33938112
J Biol Chem. 2003 Dec 26;278(52):52873-80
pubmed: 14561769
J Biol Chem. 2004 Apr 2;279(14):13488-95
pubmed: 14724270
Cell Metab. 2013 May 7;17(5):709-18
pubmed: 23623749
PLoS Genet. 2019 Dec 23;15(12):e1008548
pubmed: 31869331
J Pediatr. 2020 Feb;217:139-144
pubmed: 31732128
Nature. 2019 Nov;575(7782):395-401
pubmed: 31600774
Nat Commun. 2019 Mar 20;10(1):1287
pubmed: 30894536
J Cell Sci. 2013 Aug 15;126(Pt 16):3563-74
pubmed: 23781023
J Biol Chem. 2012 Nov 23;287(48):40131-9
pubmed: 23045528
Cell. 2013 Sep 26;155(1):160-71
pubmed: 24055366
Nat Rev Mol Cell Biol. 2019 Feb;20(2):85-101
pubmed: 30337668