Detection of Human SMPD1 by Western Blot.
Western blot shows lysates of human cerebellum tissue. PVDF membrane was probed with 1 µg/mL of Goat Anti-Human SMPD1 Antigen Affinity-purified Polyclonal Antibody (Catalog # AF5348) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # HAF019). A specific band was detected for SMPD1 at approximately 85 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 8.
Preparation and Storage
Reconstitute at 0.2 mg/mL in sterile PBS.
Reconstitution Buffer Available
The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
Stability & Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
12 months from date of receipt, -20 to -70 °C as supplied.
1 month, 2 to 8 °C under sterile conditions after reconstitution.
6 months, -20 to -70 °C under sterile conditions after reconstitution.
Sphingomyelin phosphodiesterase, also known as acid sphingomyelinase and encoded by the SMPD1 gene, is a lysosomal phosphodiesterase which belongs to the acid sphingomyelinase family (1). SMPD1 catalyzes the hydrolysis of sphingomyelin to ceramide and phosphorylcholine. Ceramide, a bioactive lipid, has emerged as an important signaling molecule involved in a variety of cellular processes such as cell differentiation, apoptosis, and proliferation (2). Activation of SMPD1 occurs by the removal, chemical modification or dimerization of its C-terminal cysteine residue (3). Deficiencies of SMPD1 result in a lysosomal storage disorder referred to as Niemann-Pick disease (4). rhSMPD1 was expressed without the last three C-terminal residues, and is therefore constitutively active.
Schuchman, E.H. et al. (1991) J. Biol. Chem. 266:8531.
Melendez, A.J. et al. (2008) Biochim. Biophys. Acta 1784:66.
Qiu, H. et al. (2003) J. Biol. Chem. 278:32744.
Smith, E.L. and Schuchman, E.H. (2008) FASEB J. 22:3419.
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