- PA3-070 - Invitrogen Antibodies SMPX antibody

Submitted references Missense mutations in small muscle protein X-linked (SMPX) cause distal myopathy with protein inclusions.

Johari M, Sarparanta J, Vihola A, Jonson PH, Savarese M, Jokela M, Torella A, Piluso G, Said E, Vella N, Cauchi M, Magot A, Magri F, Mauri E, Kornblum C, Reimann J, Stojkovic T, Romero NB, Luque H, Huovinen S, Lahermo P, Donner K, Comi GP, Nigro V, Hackman P, Udd B
Acta neuropathologica 2021 Aug;142(2):375-393

The nuclear receptor NOR-1 regulates the small muscle protein, X-linked (SMPX) and myotube differentiation.

Ferrán B, Martí-Pàmies I, Alonso J, Rodríguez-Calvo R, Aguiló S, Vidal F, Rodríguez C, Martínez-González J
Scientific reports 2016 May 16;6:25944

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Supportive validation

Submitted by: Invitrogen Antibodies (provider)
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Experimental details: Western blot analysis of SMPX in mouse heart using a polyclonal antibody (Product # PA3-070).

Supportive validation

Submitted by: Invitrogen Antibodies (provider)
Main image
Experimental details: Immunohistochemistry of SMPX in mouse cardiomyocytes

Supportive validation

Submitted by: Invitrogen Antibodies (provider)
Main image
Experimental details: Fig. 4 Immunofluorescence microscopy. a - f Immunofluorescent double staining; merged images are shown, SMPX is green and CRYAB is magenta . In control skeletal muscle (ctrl) free of neuromuscular disease, SMPX shows diffuse cytoplasmic and focal subsarcolemmal staining pattern. Muscle biopsy of F1 II.1 b shows moderate SMPX accumulation in a single fiber ( white arrowhead ), whereas in patient F2 II.2 c there is no SMPX accumulation. In probands from families F7, F8 and F9 ( d - f ), multiple SMPX-positive sarcoplasmic inclusions are present in several fibers. In F8 III.2 ( e ) and F9 II.1 ( f ), separate myofibrillar CRYAB accumulation is observed ( white arrowheads ), which does not co-localize with SMPX labeling. g - i Double immunostaining of F6 II.1. Single channel and merged images are shown; SMPX is green , vinculin is magenta . SMPX-positive protein inclusions ( g ) are also positive for vinculin ( h ), merged image ( i ). j - l ; serial sections Myofibrillar accumulation ( arrow ) is positive for myotilin ( j ), and also for the CASA proteins BAG3 ( k ) and HSPB8 ( l ). m - n ; serial sections) Several (atrophic) fibers show sarcoplasmic up-regulation of both BAG3 ( m ) and ( n ) HSPB8. Scale bars = 100 um

Supportive validation

Submitted by: Invitrogen Antibodies (provider)
Main image
Experimental details: Fig. 5 SMPX aggregation in patient muscle. a Congo red staining in fluorescence microscopy using Texas red filter showing abundant positive sarcoplasmic inclusions in muscle biopsy from patient F9 II.1 (scale bar = 50 um). b Confocal sections of a muscle biopsy from patient F8 III.1. Immunostaining of SMPX or myotilin ( green ) combined with Amytracker 680 (AT680, magenta ) shows Amytracker fluorescence in SMPX inclusions. A serial section of the same fiber shows no signal when Amytracker is omitted (BAG3/ctrl), showing that the protein inclusions are not autofluorescent. c - e EM findings of patient F9 II.1. c One large subsarcolemmal protein inclusion with accumulation of filamentous amyloid-like material ( black arrowhead ). d Myeloid bodies ( white arrowhead ) surrounding sarcoplasmic inclusions ( black arrowhead ). e Two classic cytoplasmic bodies (CB) with radiating filaments, together with six individual sarcoplasmic inclusions ( black arrowheads ) and nucleus (N)

PA3-070

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