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Product Name Adenylosuccinate Lyase Chinese Name 腺苷酸琥珀酸裂解酶抗体 Alias Adenylosuccinase; Adenylosuccinate lyase; ADSL; AMPS; ASase; ASL; PUR8_HUMAN. Research Area Cell biology Signal transduction Immunogen Species Rabbit Clonality Polyclonal React Species Human, Mouse, Rat, (predicted: Dog, Pig, Cow, Horse, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 55kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Adenylosuccinate Lyase: 185-280/484 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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
Omim: 608222 Human
SwissProt: P30566 Human
SwissProt: P54822 Mouse
Unigene: 75527 Human
Unigene: 38151 Mouse
Product Picture 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 (SL6352R) 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 (SL6352R) 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 (SL6352R) 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 (SL6352R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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