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Acid sphingomyelinase Rabbit pAb (bs-6318R)  
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产品编号 bs-6318R
英文名称 Acid sphingomyelinase Rabbit pAb
中文名称 酸性神经鞘磷脂酶抗体
别    名 Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase.  
Specific References  (4)     |     bs-6318R has been referenced in 4 publications.
[IF=6.126] Peñate T et al. Lipid-Iron Nanoparticle with a Cell Stress Release Mechanism Combined with a Local Alternating Magnetic Field Enables Site-Activated Drug ReleaseCancers (Basel).2020 Dec 14;12(12):3767.  IHC ;  Mouse.  
[IF=6.081] Tuula Penate Medina. et al. Utilizing Sphingomyelinase Sensitizing Liposomes in Imaging Intestinal Inflammation in Dextran Sulfate Sodium-Induced Murine Colitis. Biomedicines. 2022 Feb;10(2):413  IHC ;  Human.  
[IF=6.02] Bodas M et al. Autophagy augmentation alleviates cigarette smoke-induced CFTR-dysfunction, ceramide-accumulation and COPD-emphysema pathogenesis.(2018) Free Radic Biol Med.131:81-97.  FCM ;  Human.  
[IF=2.87] Anastasia M. Ravodina. et al. Facile Cholesterol Loading with a New Probe ezFlux Allows for Streamlined Cholesterol Efflux Assays. Acs Omega. 2020;5(36):23289–23298  WB ;  Mouse.  
研究领域 细胞生物  神经生物学  信号转导  细胞凋亡  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Human,Mouse,Rat (predicted: Rabbit,Pig,Cow,Dog)
产品应用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=2ug/Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 64 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Acid sphingomyelinase: 201-300/629 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.
Involvement in disease: Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) ; also known as Niemann-Pick disease classical infantile form. It is 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, mental retardation, 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.

Function:
Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.

Subunit:
Monomer.

Subcellular Location:
Lysosome.

DISEASE:
Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) [MIM:257200]; also known as Niemann-Pick disease classical infantile form. It is 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, mental retardation, 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.
Defects in SMPD1 are the cause of Niemann-Pick disease type B (NPDB) [MIM:607616]; also known as Niemann-Pick disease visceral form. It is 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.

Similarity:
Belongs to the acid sphingomyelinase family.
Contains 1 saposin B-type domain.

SWISS:
P17405

Gene ID:
6609

Database links:

Entrez Gene: 505097 Cow

Entrez Gene: 485334 Dog

Entrez Gene: 100720041 Guinea pig

Entrez Gene: 6609 Human

Entrez Gene: 20597 Mouse

Entrez Gene: 100353898 Rabbit

Entrez Gene: 308909 Rat

Omim: 607608 Human

SwissProt: Q0VD19 Cow

SwissProt: P17405 Human

SwissProt: Q04519 Mouse

Unigene: 498173 Human

Unigene: 4628 Mouse

Unigene: 485064 Mouse

Unigene: 18277 Rat



ASM酸性神经鞘磷脂酶是ASMase神经鞘磷脂酶最重要的一个亚型,是细胞膜的重要组成成分。ASM在细胞凋亡、调节肿瘤细胞生长、参与Fas信号系统传递等方面均可发挥重要作用。
产品图片
Sample: HepG2(human) cell Lysate at 30 ug MCF-7(human) cell Lysate at 30 ug A431(human) cell Lysate at 30 ug Hale(human) cell Lysate at 30 ug Primary: Anti- Acid sphingomyelinase (bs-6318R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 64kD Observed band size: 69 kD
Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Acid sphingomyelinase) Polyclonal Antibody, Unconjugated (bs-6318R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Blank control:A431. Primary Antibody (green line): Rabbit Anti-Acid sphingomyelinase antibody (bs-6318R) Dilution: 2μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody : Goat anti-rabbit IgG-PE Dilution: 1μg /test. Protocol The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 0.1% PBST for 20 min at room temperature.The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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