Mouse Anti-SMPD1 Recombinant Antibody (CBXS-5102) (CBMAB-S2320-CQ)

Mouse

Human

CBXS-5102

IgG2a, κ

ELISA, WB

Human

IgG2a, κ

Monoclonal

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Liquid

PBS, pH 7.2

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

sphingomyelin phosphodiesterase 1, acid lysosomal

The protein encoded by this gene is a lysosomal acid sphingomyelinase that converts sphingomyelin to ceramide. The encoded protein also has phospholipase C activity. Defects in this gene are a cause of Niemann-Pick disease type A (NPA) and Niemann-Pick disease type B (NPB). Multiple transcript variants encoding different isoforms have been identified.

Sphingomyelin Phosphodiesterase 1; Acid Sphingomyelinase; Sphingomyelin Phosphodiesterase 1, Acid Lysosomal; ASMASE; ASM; Sphingomyelin Phosphodiesterase; EC 3.1.4.12; NPD;

Converts sphingomyelin to ceramide (PubMed:1840600, PubMed:18815062, PubMed:27659707, PubMed:25920558, PubMed:25339683, PubMed:33163980, PubMed:12563314).
Exists as two enzymatic forms that arise from alternative trafficking of a single protein precursor, one that is targeted to the endolysosomal compartment, whereas the other is released extracellularly (PubMed:21098024, PubMed:9660788, PubMed:20807762).
However, in response to various forms of stress, lysosomal exocytosis may represent a major source of the secretory form (PubMed:20530211, PubMed:12563314, PubMed:20807762, PubMed:9393854, PubMed:22573858).
In the lysosomes, converts sphingomyelin to ceramide (PubMed:20807762, PubMed:21098024).
Plays an important role in the export of cholesterol from the intraendolysosomal membranes (PubMed:25339683).
Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol (PubMed:25339683).
Modulates stress-induced apoptosis through the production of ceramide (PubMed:8706124).
When secreted, modulates cell signaling with its ability to reorganize the plasma membrane by converting sphingomyelin to ceramide (PubMed:12563314, PubMed:20807762, PubMed:17303575).
Secreted form is increased in response to stress and inflammatory mediators such as IL1B, IFNG or TNF as well as upon infection with bacteria and viruses (PubMed:12563314, PubMed:20807762, PubMed:9393854).
Produces the release of ceramide in the outer leaflet of the plasma membrane playing a central role in host defense (PubMed:12563314, PubMed:20807762, PubMed:9393854).
Ceramide reorganizes these rafts into larger signaling platforms that are required to internalize P. aeruginosa, induce apoptosis and regulate the cytokine response in infected cells (PubMed:12563314).
In wounded cells, the lysosomal form is released extracellularly in the presence of Ca2+ and promotes endocytosis and plasma membrane repair (PubMed:20530211).
Sphingomyelin phosphodiesterase, processed form
This form is generated following cleavage by CASP7 in the extracellular milieu in response to bacterial infection (PubMed:21157428).
It shows increased ability to convert sphingomyelin to ceramide and promotes plasma membrane repair (By similarity).
Plasma membrane repair by ceramide counteracts the action of gasdermin-D (GSDMD) perforin (PRF1) pores that are formed in response to bacterial infection (By similarity).
(Microbial infection) Secretion is activated by bacteria such as P. aeruginos, N. gonorrhoeae and others, this activation results in the release of ceramide in the outer leaflet of the plasma membrane which facilitates the infection.
(Microbial infection) Secretion is activated by human coronaviruses SARS-CoV and SARS-CoV-2 as well as Zaire ebolavirus, this activation results in the release of ceramide in the outer leaflet of the plasma membrane which facilitates the infection.
Isoform 2
Lacks residues that bind the cofactor Zn2+ and has no enzyme activity.
Isoform 3
Lacks residues that bind the cofactor Zn2+ and has no enzyme activity.

Biological Process cellular response to calcium ionManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process cellular response to UVManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process ceramide biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process cholesterol metabolic processIEA:Ensembl
Biological Process glycosphingolipid metabolic processTAS:Reactome
Biological Process negative regulation of MAP kinase activityManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process nervous system developmentManual Assertion Based On ExperimentTAS:ProtInc
Biological Process plasma membrane repairManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process positive regulation of apoptotic processManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process positive regulation of endocytosisManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process positive regulation of protein dephosphorylationManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process positive regulation of viral entry into host cellManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process response to cocaineIEA:Ensembl
Biological Process response to interleukin-1Manual Assertion Based On ExperimentIDA:UniProtKB
Biological Process response to ionizing radiationManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process response to tumor necrosis factorManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process response to type I interferonManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process response to virusManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process response to xenobiotic stimulusIEA:Ensembl
Biological Process signal transductionManual Assertion Based On ExperimentTAS:ProtInc
Biological Process sphingomyelin catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process sphingomyelin metabolic processManual Assertion Based On ExperimentTAS:ProtInc
Biological Process termination of signal transductionManual Assertion Based On ExperimentIMP:BHF-UCL
Biological Process viral entry into host cellManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process wound healingManual Assertion Based On ExperimentIDA:UniProtKB

Lysosome
Lipid droplet
Secreted
The secreted form is induced in a time- and dose-dependent by IL1B and TNF as well as stress and viral infection. This increase of the secreted form seems to be due to exocytosis of the lysosomal form and is Ca2+-dependent (PubMed:20807762, PubMed:22573858, PubMed:20530211).
Secretion is dependent of phosphorylation at Ser-510 (PubMed:17303575).
Secretion is induced by inflammatory mediators such as IL1B, IFNG or TNF as well as infection with bacteria and viruses (PubMed:12563314, PubMed:20807762).
Sphingomyelin phosphodiesterase, processed form
Secreted, extracellular space
This form is generated following cleavage by CASP7.

Niemann-Pick disease A (NPDA):
An early-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Niemann-Pick disease type A is a primarily neurodegenerative disorder characterized by onset within the first year of life, intellectual disability, digestive disorders, failure to thrive, major hepatosplenomegaly, and severe neurologic symptoms. The severe neurological disorders and pulmonary infections lead to an early death, often around the age of four. Clinical features are variable. A phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B.
Niemann-Pick disease B (NPDB):
A late-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Clinical signs involve only visceral organs. The most constant sign is hepatosplenomegaly which can be associated with pulmonary symptoms. Patients remain free of neurologic manifestations. However, a phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B. In Niemann-Pick disease type B, onset of the first symptoms occurs in early childhood and patients can survive into adulthood.

Proteolytically processed (PubMed:21098024, PubMed:9030779).
Mature lysosomal form arises from C-terminal proteolytic processing of pro-sphingomyelin phosphodiesterase (PubMed:21098024).
Sphingomyelin phosphodiesterase, processed form
This form is generated following cleavage by CASP7 in the extracellular milieu (PubMed:21157428).
It shows increased activity (By similarity).
Both lysosomal and secreted forms are glycosylated but they show a differential pattern of glycosylation.
Phosphorylated at Ser-510 by PRKCD upon stress stimuli. Phosphorylation is required for secretion.