PA3-070

antibody from Invitrogen Antibodies

Targeting: SMPX Chisel, Csl, DFN6, DFNX4

Immunohistochemistry

Antibody data

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Validation data

Product number: PA3-070 - Provider product page
Provider: Invitrogen Antibodies
Product name: SMPX Polyclonal Antibody
Antibody type: Polyclonal
Antigen: Other
Description: PA3-070 detects SMPX from human, mouse, and rat samples. PA3-070 has been successfully used in Western blot, immunocytochemistry, immunohistochemistry (paraffin) procedures. By Western blot, this antibody detects a 9 kDa protein corresponding to mouse SMPX. The PA3-070 antigen is the COOH-terminal amino acids of mouse SMPX (66–84) NLSEIQNVKSELKFVPKGE coupled to KLH.
Reactivity: Human, Mouse, Rat
Host: Rabbit
Isotype: IgG
Vial size: 100 µL
Concentration: Conc. Not Determined
Storage: -20° C, Avoid Freeze/Thaw Cycles

SMPX protein structure - PA3-070 shown in red.

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)