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Rabbit Anti-Adenylosuccinate Lyase  antibody (bs-6352R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-6352R
英文名称 Adenylosuccinate Lyase
中文名称 腺苷酸琥珀酸裂解酶抗体
别    名 Adenylosuccinase; Adenylosuccinate lyase; ADSL; AMPS; ASase; ASL; PUR8_HUMAN.  
研究领域 细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human,Mouse,Rat (predicted: Dog,Pig,Cow,Horse)
产品应用 WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 55kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Adenylosuccinate Lyase: 185-280/484 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C 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 Adenylsuccinate lyase is involved in both de novo synthesis of purines and formation of adenosine monophosphate from inosine monophosphate. It catalyzes two reactions in AMP biosynthesis: the removal of a fumarate from succinylaminoimidazole carboxamide (SAICA) ribotide to give aminoimidazole carboxamide ribotide (AICA) and removal of fumarate from adenylosuccinate to give AMP. Adenylosuccinase deficiency results in succinylpurinemic autism, psychomotor retardation, and , in some cases, growth retardation associated with muscle wasting and epilepsy. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].

Tissue Specificity:
Ubiquitously expressed. Both isoforms are produced by all tissues. Isoform 2 is 10-fold less abundant than isoform 1.

DISEASE:
Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency) . ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present.

Similarity:
Belongs to the lyase 1 family. Adenylosuccinate lyase subfamily.

SWISS:
P30566

Gene ID:
158

Database links:

Entrez Gene: 158 Human

Entrez Gene: 11564 Mouse

Entrez Gene: 315150 Rat

Omim: 608222 Human

SwissProt: P30566 Human

SwissProt: P54822 Mouse

Unigene: 75527 Human

Unigene: 38151 Mouse



产品图片
Sample:
Lane 1: Stomach (Mouse) Lysate at 40 ug
Lane 2: Spleen (Mouse) Lysate at 40 ug
Lane 3: Jurkat (Human) Cell Lysate at 30 ug
Lane 4: HL60 (Human) Cell Lysate at 30 ug
Lane 5: 293T (Human) Cell Lysate at 30 ug
Primary: Anti-Adenylosuccinate Lyase (bs-6352R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 55/48 kD
Observed band size: 50 kD
Paraformaldehyde-fixed, paraffin embedded (Human kidney); 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 (Adenylosuccinate Lyase) Polyclonal Antibody, Unconjugated (bs-6352R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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 (Adenylosuccinate Lyase) Polyclonal Antibody, Unconjugated (bs-6352R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (mouse 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 (Adenylosuccinate Lyase) Polyclonal Antibody, Unconjugated (bs-6352R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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