N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 Proteins (ASAH1)

ASAH1 encodes a heterodimeric protein consisting of a nonglycosylated alpha subunit and a glycosylated beta subunit that is cleaved to the mature enzyme posttranslationally. Additionally we are shipping N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 Antibodies (80) and N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 Kits (6) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
ASAH1 427 Q13510
ASAH1 11886 Q9WV54
ASAH1 84431 Q6P7S1
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Top N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 Proteins at antibodies-online.com

Showing 9 out of 12 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
Insect Cells Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 50 Days
$6,749.58
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Insect Cells Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 50 Days
$6,749.58
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Wheat germ Human GST tag 2 μg Log in to see 11 to 12 Days
$230.67
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HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Log in to see 11 Days
$888.80
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Yeast Rat His tag   1 mg Log in to see 60 to 71 Days
$3,026.83
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Yeast Chimpanzee His tag   1 mg Log in to see 60 to 71 Days
$3,026.83
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Yeast Cow His tag   1 mg Log in to see 60 to 71 Days
$3,030.50
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Yeast Caenorhabditis elegans His tag   1 mg Log in to see 60 to 71 Days
$3,036.00
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Escherichia coli (E. coli) Human His tag   100 μg Log in to see 15 to 18 Days
$656.00
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ASAH1 Proteins by Origin and Source

Origin Expressed in Conjugate
Human , , ,
, ,
Mouse (Murine)

Rat (Rattus)

Top referenced N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 Proteins

  1. Human ASAH1 Protein expressed in Wheat germ - ABIN4948350 : Sabel, Liu, Griffith, He, Xie, Lubman: Clinical utility of serum autoantibodies detected by protein microarray in melanoma. in International journal of proteomics 2011 (PubMed)

More Proteins for N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) Interaction Partners

Human N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) interaction partners

  1. The expression of ASAH1 was associated significantly with lymph nodes metastasis, indicating that ASAH1 may serve as a biomarker to predict patients' lymph nodes status in breast cancers.

  2. We also identified ASAH1 as a new target of MITF, thereby involving MITF in the regulation of sphingolipid metabolism. Together, our findings provide new cues to the mechanisms underlying the phenotypic plasticity of melanoma cells and identify new anti-metastatic targets.

  3. Acid ceramidase ablation blocks cell cycle progression and accelerates senescence. Importantly, ASAH1-null cells also lose the ability to form cancer-initiating cells and to undergo self-renewal, which is suggestive of a key role for acid ceramidase in maintaining malignancy and self-renewal of invasive melanoma cells.

  4. Autocleavage of ASAH1 triggers a conformational change exposing a hydrophobic channel leading to the active site.

  5. results demonstrate a tissue-specific role for AC in regulating neutrophilic inflammation and cytokine production.

  6. Acid ceramidase plays a critical role in acute myeloid leukemia cell survival via regulation of both sphingolipid levels and Mcl-1.

  7. Collectively, our data show the novel discovery of anti-inflammatory and anti-apoptotic effects of acid ceramidase in host cells exposed to periodontal bacteria, and the attenuation of the expression of host-protective acid ceramidase in periodontal lesions.

  8. we describe an individual with a sporadic atypical spinal muscular atrophy, in whom clinical DNA sequencing reported one pathogenic ASAH1 mutation .Transcriptome sequencing on patient leukocytes identified a highly significant and atypical ASAH1 isoform not explained by c.458A>G(p<10(-16) ).

  9. ASAH1 variant Leu401Pro co-segregates with keloid phenotype in a large Yoruba family.

  10. We report an atypical presentation of Farber disease with her pathology and associated genetic defect. This case expands the phenotypic spectrum of Farber disease to include novel mutations of ASAH1, which pose a diagnostic challenge.

  11. This study describes for the first time the association between ASAH1 variants and an adult SMA phenotype with no myoclonic epilepsy nor death in early age, thus expanding the phenotypic spectrum of ASAH1-related SMA.

  12. Our findings indicate that hypomorphic mutations in ASAH1 may result in an osteoarticular phenotype with a juvenile phase resembling rheumatoid arthritis that evolves to osteolysis as the final stage in the absence of neurologic signs. This observation delineates a novel type of recessively inherited peripheral osteolysis and illustrates the long-term skeletal manifestations of acid ceramidase deficiency (Farber's disease

  13. The present report describes a 9-year-old girl with novel clinical phenotype of a patient with polyarticular arthritis followed by symptoms of SMA due to acid ceramidase deficiency. Whole exome sequencing identified compound heterozygous pathogenic mutation in the N-acylsphingosine amidohydrolase 1 gene.

  14. Data suggest up-regulation of ASAH1 activity by androgen in androgen-sensitive prostate cancer cells (not other cancer cells) is due to prolonged stability of ASAH1 by androgen-stimulated induction of USP2 (ubiquitin specific peptidase 2) expression.

  15. AC-controlled sphingolipid metabolism may play an important role in the control of melanoma proliferation

  16. Genetic or pharmacological acid ceramidase inhibition promotes cisplatin cytotoxicity in head and neck tumor cells.

  17. high ASAH1 expression is generally associated with an improved prognosis in invasive breast cancer independent of adjuvant treatment and could also be valuable as prognostic factor for pre-invasive DCIS.

  18. novel ASAH1 mutations affecting polypyrimidine tract deletion, and exon skipping and resulting in Farber lipogranulomatosis

  19. Acid ceramidase promotes nuclear export of PTEN through sphingosine 1-phosphate mediated Akt signaling.

  20. Depletion of both Akt2 and ASAH1 is much more potent than depleting each alone at inhibiting neoplastic cell viability/proliferation and invasion.

Mouse (Murine) N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) interaction partners

  1. results demonstrate a tissue-specific role for AC in regulating neutrophilic inflammation and cytokine production.

  2. Low ASAH1 expression is associated with Chronic lung injury.

  3. cells deficient in acid ceramidase (aCDase) also exhibited defects in CCL5 induction, whereas cells deficient in sphingosine kinase-1 and -2 exhibited higher levels of CCL5.

  4. Mutagenesis leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes.

  5. biochemistry of acid ceramidase reaction with acid sphingomyelinase

  6. Acid and neutral sphingomyelinase, ceramide synthase, and acid ceramidase have roles in cutaneous aging

  7. These results provide the first characterization of the Acid Ceremidase promoter from any species and demonstrate that Kruppel-like factor 6 (KLF6) is one transcription factor involved in the regulation of AC gene expression.

  8. acid ceramidase is an essential factor required for embryo survival that functions by removing ceramide from the newly formed embryos, thus inhibiting the default apoptosis pathway

  9. AC is a critical regulator of prostate cancer progression by affecting not only tumor cell proliferation and migration but also responses to drug therapy

N-Acylsphingosine Amidohydrolase (Acid Ceramidase) 1 (ASAH1) Protein Profile

Protein Summary

This gene encodes a heterodimeric protein consisting of a nonglycosylated alpha subunit and a glycosylated beta subunit that is cleaved to the mature enzyme posttranslationally. The encoded protein catalyzes the synthesis and degradation of ceramide into sphingosine and fatty acid. Mutations in this gene have been associated with a lysosomal storage disorder known as Farber disease. Multiple transcript variants encoding several distinct isoforms have been identified for this gene.

Gene names and symbols associated with ASAH1

  • N-acylsphingosine amidohydrolase 1 (ASAH1)
  • N-acylsphingosine amidohydrolase 1 (Asah1)
  • N-acylsphingosine amidohydrolase (acid ceramidase) 1 (ASAH1)
  • N-acylsphingosine amidohydrolase (acid ceramidase) 1 L homeolog (asah1.L)
  • N-acylsphingosine amidohydrolase (acid ceramidase) 1a (asah1a)
  • Acid ceramidase (asah-1)
  • 2310081N20Rik protein
  • AC protein
  • ACDase protein
  • Asah protein
  • ASAH1 protein
  • MGC82286 protein
  • PHP protein
  • PHP32 protein
  • SMAPME protein
  • zgc:101637 protein

Protein level used designations for ASAH1

acid CDase , acid ceramidase , acylsphingosine deacylase , putative 32 kDa heart protein , ACDase , Acylsphingosine deacylase , N-acylsphingosine amidohydrolase 1 , AC , N-acylsphingosine amidohydrolase (acid ceramidase) 1

GENE ID SPECIES
427 Homo sapiens
11886 Mus musculus
464022 Pan troglodytes
84431 Rattus norvegicus
422727 Gallus gallus
447327 Xenopus laevis
450068 Danio rerio
482897 Canis lupus familiaris
510620 Bos taurus
703699 Macaca mulatta
100171626 Pongo abelii
100223185 Taeniopygia guttata
100338209 Oryctolagus cuniculus
173120 Caenorhabditis elegans
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