- MA5-34796 - Invitrogen Antibodies ADAMTS13 antibody

Submitted references A spoonful of L-fucose-an efficient therapy for GFUS-CDG, a new glycosylation disorder.

Feichtinger RG, Hüllen A, Koller A, Kotzot D, Grote V, Rapp E, Hofbauer P, Brugger K, Thiel C, Mayr JA, Wortmann SB
EMBO molecular medicine 2021 Sep 7;13(9):e14332

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Supportive validation

Submitted by: Invitrogen Antibodies (provider)
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Experimental details: Western blot analysis of ADAMTS13 in SiHa cell (1), PC-3M cell (2) and A549 cell lysates. Samples were incubated with ADAMTS13 monoclonal antibody (Product # MA5-34796), at a dilution of 1:500.

Supportive validation

Submitted by: Invitrogen Antibodies (provider)
Main image
Experimental details: Flow cytometry of ADAMTS13 in HepG2 cells (purple) compared with an unlabelled control (cells without incubation with primary antibody; yellow). Samples were incubated with ADAMTS13 monoclonal antibody (Product # MA5-34796) at a dilution of 1:100, followed by Alexa Fluor 488-conjugated goat anti-rabbit IgG.

Supportive validation

Submitted by: Invitrogen Antibodies (provider)
Main image
Experimental details: EV3 Figure Effects of GFUS deficiency on the expression of fucosyltransferases and ADAMTS13 Western blot analysis of FUT8, POFUT1 and POFUT2. Expression of the proteins was analysed by western blotting on a 10% SDS-PAGE with cytosolic fractions derived from control and patient fibroblasts. Data were obtained from fibroblasts; n = 3, for statistics an unpaired t -test was performed. qPCR studies on FUT8, POFUT1 and POFUT2 . mRNA expression revealed a significantly increased transcript level of FUT8 (2.79 fold change; +- 1,10 fold change; * P = 0.0498) normalized to a control. Expression of POFUT1 and POFUT2 was not changed significantly. Data were obtained from fibroblasts; n = 9; experiment was independently repeated three times, for statistics an ANOVA was performed. Expression of ADAMTS13 in sera. The amount of ADAMTS13 secreted into sera of controls and the patient before and after 8 weeks of L-fucose treatment was detected by western blot and normalized to transferrin. Data were obtained from serum; n = 12 for controls. Data information: * P < 0.05; ** P < 0.01. Bars and error bars represent mean +- SD. Exact P -values are reported in the results part.

Supportive validation

Submitted by: Invitrogen Antibodies (provider)
Main image
Experimental details: Figure EV3 Effects of GFUS deficiency on the expression of fucosyltransferases and ADAMTS13 Western blot analysis of FUT8, POFUT1 and POFUT2. Expression of the proteins was analysed by western blotting on a 10% SDS-PAGE with cytosolic fractions derived from control and patient fibroblasts. Data were obtained from fibroblasts; n = 3, for statistics an unpaired t -test was performed. qPCR studies on FUT8, POFUT1 and POFUT2 . mRNA expression revealed a significantly increased transcript level of FUT8 (2.79 fold change; +- 1,10 fold change; * P = 0.0498) normalized to a control. Expression of POFUT1 and POFUT2 was not changed significantly. Data were obtained from fibroblasts; n = 9; experiment was independently repeated three times, for statistics an ANOVA was performed. Expression of ADAMTS13 in sera. The amount of ADAMTS13 secreted into sera of controls and the patient before and after 8 weeks of L-fucose treatment was detected by western blot and normalized to transferrin. Data were obtained from serum; n = 12 for controls. Data information: * P < 0.05; ** P < 0.01. Bars and error bars represent mean +- SD. Exact P -values are reported in the results part.

MA5-34796

Antibody data