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Mouse Anti-ASAH1 Recombinant Antibody (23/Acid Ceramidase) (CBMAB-A3701-YC)

Provided herein is a Mouse monoclonal antibody against Human N-Acylsphingosine Amidohydrolase 1. The antibody can be used for immunoassay techniques, such as WB.
See all ASAH1 antibodies
Published Data
Mouse Anti-ASAH1 Recombinant Antibody (23/Acid Ceramidase) CBMAB-A3701-YC in WB
Western blot analysis of the levels of ASAH1 and loading control GAPDH in cells harvested after treatment with either vehicle (DMSO) or the protein synthesis inhibitor cycloheximide (CHX) for the times shown. ...View More
Munk, R., Anerillas, C., Rossi, M., Tsitsipatis, D., Martindale, J. L., Herman, A. B., ... & Abdelmohsen, K. (2021). Acid ceramidase promotes senescent cell survival. Aging (albany NY), 13(12), 15750.
Mouse Anti-ASAH1 Recombinant Antibody (23/Acid Ceramidase) CBMAB-A3701-YC in WB
Relative expression levels of acid ceramidase (AC) in pancreatic cancer cells. Western blot analysis. ...View More
Taniai, T., Shirai, Y., Shimada, Y., Hamura, R., Yanagaki, M., Takada, N., ... & Ikegami, T. (2021). Inhibition of acid ceramidase elicits mitochondrial dysfunction and oxidative stress in pancreatic cancer cells. Cancer Science, 112(11), 4570-4579.

Summary

Host Animal
Mouse
Specificity
Human, Dog
Clone
23/Acid Ceramidase
Antibody Isotype
IgG1, κ
Application
WB

Basic Information

Immunogen
Human Acid Ceramidase, aa 88-182.
Specificity
Human, Dog
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:100-1:1,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, 0.1% gelatin
Preservative
< 0.1% sodium azide
Concentration
0.2 mg/ml
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
N-acylsphingosine amidohydrolase (acid ceramidase) 1
Introduction
ASAH1 is a member of the acid ceramidase family of proteins.
Entrez Gene ID
Human427
Dog482897
UniProt ID
HumanQ13510
DogF1PWA9
Alternative Names
N-Acylsphingosine Amidohydrolase 1; Acylsphingosine Deacylase; Putative 32 KDa Heart Protein; Acid Ceramidase; EC 3.5.1.23; Acid CDase; ACDase; PHP32;
Function
Lysosomal ceramidase that hydrolyzes sphingolipid ceramides into sphingosine and free fatty acids at acidic pH (PubMed:10610716, PubMed:7744740, PubMed:15655246, PubMed:11451951).
Ceramides, sphingosine, and its phosphorylated form sphingosine-1-phosphate are bioactive lipids that mediate cellular signaling pathways regulating several biological processes including cell proliferation, apoptosis and differentiation (PubMed:10610716).
Has a higher catalytic efficiency towards C12-ceramides versus other ceramides (PubMed:7744740, PubMed:15655246).
Also catalyzes the reverse reaction allowing the synthesis of ceramides from fatty acids and sphingosine (PubMed:12764132, PubMed:12815059).
For the reverse synthetic reaction, the natural sphingosine D-erythro isomer is more efficiently utilized as a substrate compared to D-erythro-dihydrosphingosine and D-erythro-phytosphingosine, while the fatty acids with chain lengths of 12 or 14 carbons are the most efficiently used (PubMed:12764132).
Has also an N-acylethanolamine hydrolase activity (PubMed:15655246).
By regulating the levels of ceramides, sphingosine and sphingosine-1-phosphate in the epidermis, mediates the calcium-induced differentiation of epidermal keratinocytes (PubMed:17713573).
Also indirectly regulates tumor necrosis factor/TNF-induced apoptosis (By similarity).
By regulating the intracellular balance between ceramides and sphingosine, in adrenocortical cells, probably also acts as a regulator of steroidogenesis (PubMed:22261821).
Isoform 2: May directly regulate steroidogenesis by binding the nuclear receptor NR5A1 and negatively regulating its transcriptional activity.
Biological Process
Cellular response to tumor necrosis factor Source: UniProtKB
Ceramide biosynthetic process Source: UniProtKB
Ceramide catabolic process Source: UniProtKB
Fatty acid metabolic process Source: InterPro
Glycosphingolipid metabolic process Source: Reactome
Keratinocyte differentiation Source: UniProtKB
Negative regulation of nucleic acid-templated transcription Source: UniProtKB
Neutrophil degranulation Source: Reactome
Regulation of programmed necrotic cell death Source: UniProtKB
Regulation of steroid biosynthetic process Source: UniProtKB
Sphingosine biosynthetic process Source: UniProtKB
Cellular Location
Lysosome; Secreted. Secretion is extremely low and localization to lysosomes is mannose-6-phosphate receptor-dependent.
Isoform 2: Cytoplasm; Nucleus. A localization to the nucleus and the cytoplasm has also been reported for ASAH1, most probably for isoforms devoid of a signal peptide.
Involvement in disease
Farber lipogranulomatosis (FRBRL): An autosomal recessive lysosomal storage disorder characterized by subcutaneous lipid-loaded nodules, excruciating pain in the joints and extremities, and marked accumulation of ceramide in lysosomes. Disease severity is variable. The most severe disease subtype is a rare neonatal form with death occurring before 1 year of age.
Spinal muscular atrophy with progressive myoclonic epilepsy (SMAPME): An autosomal recessive neuromuscular disorder characterized by childhood onset of motor deficits and progressive myoclonic seizures, after normal developmental milestones. Proximal muscle weakness and generalized muscular atrophy are due to degeneration of spinal motor neurons. Myoclonic epilepsy is generally resistant to conventional therapy. The disease course is progressive and leads to respiratory muscle involvement and severe handicap or early death from respiratory insufficiency.
PTM
N-glycosylated.
Proteolytically cleaved into two chains alpha and beta that remain associated via a disulfide bond (PubMed:7744740, PubMed:11451951, PubMed:30525581, PubMed:29692406). Cleavage gives rise to a conformation change that activates the enzyme. The same catalytic Cys residue mediates the autoproteolytic cleavage and subsequent hydrolysis of lipid substrates (PubMed:30525581, PubMed:29692406). The beta chain may undergo an additional C-terminal processing (PubMed:12815059).

Malvi, P., Janostiak, R., Nagarajan, A., Zhang, X., & Wajapeyee, N. (2021). N-acylsphingosine amidohydrolase 1 promotes melanoma growth and metastasis by suppressing peroxisome biogenesis-induced ROS production. Molecular metabolism, 48, 101217.

Su, Y., Yang, L., Li, Z., Wang, W., Xing, M., Fang, Y., ... & Cui, D. (2021). The interaction of ASAH1 and NGF gene involving in neurotrophin signaling pathway contributes to schizophrenia susceptibility and psychopathology. Progress in Neuro-Psychopharmacology and Biological Psychiatry, 104, 110015.

Brooks, B. M., Yeh, C. D., Beers, J., Liu, C., Cheng, Y. S., Gorshkov, K., ... & Chen, C. Z. (2021). Generation of an induced pluripotent stem cell line (TRNDi030-A) from a patient with Farber disease carrying a homozygous p. Y36C (c. 107 A> G) mutation in ASAH1. Stem Cell Research, 53, 102387.

Elsea, S. H., Solyom, A., Martin, K., Harmatz, P., Mitchell, J., Lampe, C., ... & Ferreira, C. R. (2020). ASAH1 pathogenic variants associated with acid ceramidase deficiency: Farber disease and spinal muscular atrophy with progressive myoclonic epilepsy. Human Mutation, 41(9), 1469-1487.

Sugano, E., Edwards, G., Saha, S., Wilmott, L. A., Grambergs, R. C., Mondal, K., ... & Mandal, N. (2019). Overexpression of acid ceramidase (ASAH1) protects retinal cells (ARPE19) from oxidative stress [S]. Journal of lipid research, 60(1), 30-43.

Santos-Cortez, R. L. P., Hu, Y., Sun, F., Benahmed-Miniuk, F., Tao, J., Kanaujiya, J. K., ... & Reichenberger, E. J. (2017). Identification of ASAH1 as a susceptibility gene for familial keloids. European Journal of Human Genetics, 25(10), 1155-1161.

Sugano, E., Nawajes, M. A., Tabata, K., Tamai, M., & Tomita, H. (2017). Protective effect of N-Acylsphingosine Amidohydrolase 1 (acid Ceramidase) in RPE cells against oxidative stress. Investigative Ophthalmology & Visual Science, 58(8), 5354-5354.

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For research use only. Not intended for any clinical use.

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