Table 1 Various types of anti-mitochondrial antibodies implicated in human diseases.

From: Anti-mitochondrial autoantibodies in systemic lupus erythematosus and their association with disease manifestations

Type of anti-mitochondrial antibody:

Molecular target(s):

Localization:

Associated disease(s):

Method(s) of detection:

References:

Anti-M1

Cardiolipin

IMM

APS, SLE, secondary syphilis

IIF, ELISAa, CFT

87

Anti-M2

2-oxoacid dehydrogenase complex

IMM

PBC

IIF, ELISAa, CFT

58

Anti-M3

Unknown

OMM

Venocuran-induced PLE

IIF, CFT

55

Anti-M4

Sulfite oxidase

OMM

PBC

ELISAb, CFT

88

Anti-M5

Unknown

OMM, IMM

APS, SLE, SS, haemolytic anemia

IIF, CFT

56

Anti-M6

Monoamine oxydase B

OMM

Iproniazid-induced hepatitis

IIF, ELISAb, CFT

39, 89

Anti-M7

Sarcosine dehydrogenase

IMM

Cardiomyopathies

ELISAa–c

90

Anti-M8

Unknown

OMM

PBC

CFT

89

Anti-M9

Glycogen phosphorylase

OMM

PBC

ELISA

91, 92

  1. aELISA performed on sub-mitochondrial particles (sonicated “crude” mitochondria).
  2. bELISA performed on purified antigen.
  3. cThe exact antigen recognized by M7 antibodies is yet to fully characterize.
  4. APS, anti-phospholipid syndrome; CFT, complement fixation test; ELISA, enzyme-linked immunosorbent assay; IIF, indirect immunofluorescence on rodent and/or human tissues; IMM, inner mitochondrial membrane; OMM, outer mitochondrial membrane; PBC, primary biliary cirrhosis; PLE, pseudolupus erythematosus; SLE, systemic lupus erythematosus; SS: Sjögren syndrome.
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