Immunohistochemistry (IHC)
(Rabbit Anti-ADSL AntibodyFormalin Fixed Paraffin Embedded Tissue: Human heart TissueObserved Staining: Cytoplasmic in endothelial cells in blood vesselsPrimary Antibody Concentration: 1:100Other Working Concentrations: 1:600Secondary Antibody: Donkey anti-Rabbit-Cy3Secondary Antibody Concentration: 1:200Magnification: 20XExposure Time: 0.5 - 2.0 sec)
Rabbit ADSL Polyclonal Antibody | anti-ADSL antibody
ADSL antibody - middle region
Immunohistochemistry (IHC)
(Rabbit Anti-ADSL AntibodyFormalin Fixed Paraffin Embedded Tissue: Human heart TissueObserved Staining: Cytoplasmic in endothelial cells in blood vesselsPrimary Antibody Concentration: 1:100Other Working Concentrations: 1:600Secondary Antibody: Donkey anti-Rabbit-Cy3Secondary Antibody Concentration: 1:200Magnification: 20XExposure Time: 0.5 - 2.0 sec)
Immunohistochemistry (IHC)
(Rabbit Anti-ADSL AntibodyFormalin Fixed Paraffin Embedded Tissue: Human heart TissueObserved Staining: Cytoplasmic in endothelial cells in blood vesselsPrimary Antibody Concentration: 1:100Other Working Concentrations: 1:600Secondary Antibody: Donkey anti-Rabbit-Cy3Secondary Antibody Concentration: 1:200Magnification: 20XExposure Time: 0.5 - 2.0 sec)
Western Blot (WB)
(Sample Type: HumanSample Type: 1. Hamster CHO K1 cells (20ug)2. HeLa skin cells(100ug)3. HEK273 cells (100ug)4. HepG2 cells (100ug)5. purified human ADSL protein (40ng)Primary Dilution: 1:2000Secondary Antibody: Clean-Blot IP detection Reagent and KitSecondary Dilution: 1:2000Image Submitted By: V. Skopova, M. ZikanovaInstitute of Inherited Metabolic Disorders, Charles University in Prague and General Hopspital in Prague )
Western Blot (WB)
(Sample Type: HumanSample Type: 1. Hamster CHO K1 cells (20ug)2. HeLa skin cells(100ug)3. HEK273 cells (100ug)4. HepG2 cells (100ug)5. purified human ADSL protein (40ng)Primary Dilution: 1:2000Secondary Antibody: Clean-Blot IP detection Reagent and KitSecondary Dilution: 1:2000Image Submitted By: V. Skopova, M. ZikanovaInstitute of Inherited Metabolic Disorders, Charles University in Prague and General Hopspital in Prague )
Western Blot (WB)
(Host: RabbitTarget Name: ADSLSample Type: Human Fetal LungAntibody Dilution: 1.0ug/ml)
Western Blot (WB)
(Host: RabbitTarget Name: ADSLSample Type: Human Fetal LungAntibody Dilution: 1.0ug/ml)
Western Blot (WB)
(WB Suggested Anti-ADSL Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:62500Positive Control: Human Muscle)
Western Blot (WB)
(WB Suggested Anti-ADSL Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:62500Positive Control: Human Muscle)
Target Description: 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.
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene belongs to the lyase 1 family. It is an essential enzyme involved in purine metabolism, and catalyzes two non-sequential reactions in the de novo purine biosynthetic pathway: the conversion of succinylaminoimidazole carboxamide ribotide (SAICAR) to aminoimidazole carboxamide ribotide (AICAR) and the conversion of adenylosuccinate (S-AMP) to adenosine monophosphate (AMP). Mutations in this gene are associated with adenylosuccinase deficiency (ADSLD), a disorder marked with psychomotor retardation, epilepsy or autistic features. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Dec 2015]
Uniprot Description
ADSL: 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. Belongs to the lyase 1 family. Adenylosuccinate lyase subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 4.3.2.2; Nucleotide Metabolism - purine; Lyase; Amino Acid Metabolism - alanine, aspartate and glutamate
Chromosomal Location of Human Ortholog: 22q13.2
Cellular Component: cytosol
Molecular Function: adenylosuccinate lyase activity
Biological Process: AMP biosynthetic process; purine ribonucleoside monophosphate biosynthetic process; metabolic process; nucleobase, nucleoside and nucleotide metabolic process; purine nucleotide biosynthetic process; protein tetramerization; purine base metabolic process; 'de novo' IMP biosynthetic process
Disease: Adenylosuccinase Deficiency
Research Articles on ADSL
Similar Products
Product Notes
The ADSL adsl (Catalog #AAA3214598) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ADSL antibody - middle region reacts with Cow, Dog, Guinea Pig, Horse, Human, Mouse, Pig, Rabbit, Rat, Sheep and may cross-react with other species as described in the data sheet. AAA Biotech's ADSL can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC). Researchers should empirically determine the suitability of the ADSL adsl for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Synthetic peptide located within the following region: RVRDDLRFRG VKGTTGTQAS FLQLFEGDDH KVEQLDKMVT EKAGFKRAFI. It is sometimes possible for the material contained within the vial of "ADSL, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
