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Table 6 Neuromuscular immunohistochemistry: selected antibodies (Dubowitz et al., 2020; Dubowitz et al., 2013; Paradas et al., 2005; Dastgir et al. 2016; Udd et al. 2019)

From: Muscle biopsy essential diagnostic advice for pathologists

 

Reaction

Dystrophinopathies

 Spectrin (sarcoplasmic membrane integrity marker)

SM

 Dystrophin Rod domain (DYS1)

SM

 Dystrophin carboxy-terminal (DYS2)

SM

 Dystrophin amino-terminal (DYS3)

SM

 Utrophin

SM

 Neonatal myosin

S/ RF

Other muscular dystrophies

 Spectrin (sarcoplasmic membrane integrity marker)

SM

 Alpha-sarcoglycan

SM

 Beta-sarcoglycan

SM

 Gamma-sarcoglycan

SM

 Delta-sarcoglycan

SM

 Dysferlin

SM

 Caveolin

SM

 Telethonin

S

 Emerin

NM

 Collagen VI

EM

 Laminin alpha 5

EM

 Laminin beta 1

EM

 Laminin gamma 1 (extracellular matrix integrity marker)

EM

 Merosin (alpha-2 laminin) (80 kDa, 300 kDa)

EM

 Integrin alpha-7

SM

 Alpha-dystroglycan (VIA4, IIH6C4)

EM

 Beta-dystroglycan

SM

 nNOS (neuronal nitric oxide synthase)

SM

Inflammatory myopathies

 CD3

TL

 CD4

TL

 CD8

TL

 CD20

BL

 CD138

P

 CD45

TL/ BL

 CD68

Mac

 CD31

C

 MHC-I

C

 p62

A

 C5b-9

C

Myofibrillar myopathies

 Desmin

S

 Myotilin

S

 Beta-crystallin

S

Sporadic inclusion body myositis

 Ubiquitin

S

Myosins

 Rapid myosin

S

 Slow myosin

S

 Embryonic myosin

S

 Fetal myosin

S

  1. Reaction = immunohistochemistry reaction pattern. SM sarcoplasmic membrane, S sarcoplasm, EM extracellular matrix, NM nuclear membrane, L lymphocyte, TL T lymphocyte, BL B lymphocyte, TL/BL T and B lymphocytes, C capillary vessel wall, P plasma cell (Dastgir et al., 2016), RF regeneration fiber, Mac macrophage, A aggregates