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anti-AASS antibody :: Rabbit anti-Human AASS Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS822135 anti-AASS antibody
Unit / Price
0.03 mL  /  $155 +1 FREE 8GB USB
0.1 mL  /  $220 +1 FREE 8GB USB
0.2 mL  /  $295 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

AASS, Polyclonal Antibody

 Also Known As   

Anti-AASS Antibody

 Product Synonym Names    Alpha-aminoadipic semialdehyde synthase, mitochondrial; LKR/SDH
 Product Gene Name   

anti-AASS antibody

[Similar Products]
 Antibody/Peptide Pairs    AASS peptide (MBS824074) is used for blocking the activity of AASS antibody (MBS822135)
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    phenotype 605113
 3D Structure    ModBase 3D Structure for Q9UDR5
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 Clonality    Polyclonal
 Host    Rabbit
 Species Reactivity    Human
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 Specificity    Recognizes endogenous levels of AASS protein.
 Purity/Purification    The antibody was purified by immunogen affinity chromatography.
 Form/Format    Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.
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 Immunogen    KLH-conjugated synthetic peptide encompassing a sequence within the center region of human AASS. The exact sequence is proprietary.
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 Preparation and Storage    Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.
 Other Notes    Small volumes of anti-AASS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for anti-AASS antibody

   Rabbit polyclonal antibody to AASS
 Applications Tested/Suitable for anti-AASS antibody   

Western Blot (WB), Immunoprecipitation (IP)

 Application Notes for anti-AASS antibody    WB (1/500 - 1/1000), IP (1/10 - 1/100)
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 Western Blot (WB) of anti-AASS antibody    anti-AASS antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for AASS. It may not necessarily be applicable to this product.
 NCBI GI #    13027640
 NCBI GeneID    10157
 NCBI Accession #    NP_005754.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_005763.3 [Other Products]
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 UniProt Primary Accession #    Q9UDR5 [Other Products]
 UniProt Secondary Accession #    O95462 [Other Products]
 UniProt Related Accession #    Q9UDR5 [Other Products]
 Molecular Weight    102,132 Da
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 NCBI Official Full Name    alpha-aminoadipic semialdehyde synthase, mitochondrial
 NCBI Official Synonym Full Names    aminoadipate-semialdehyde synthase
 NCBI Official Symbol    AASS [Similar Products]
 NCBI Official Synonym Symbols   
LKRSDH; LORSDH; LKR/SDH
[Similar Products]
 NCBI Protein Information    alpha-aminoadipic semialdehyde synthase, mitochondrial; lysine-2-oxoglutarate reductase; alpha-aminoadipate semialdehyde synthase; lysine-ketoglutarate reductase /saccharopine dehydrogenase
 UniProt Protein Name    Alpha-aminoadipic semialdehyde synthase, mitochondrial
 UniProt Synonym Protein Names   
LKR/SDHIncluding the following 2 domains:Lysine ketoglutarate reductase (EC:1.5.1.8); LKR; LOR; Saccharopine dehydrogenase (EC:1.5.1.9); SDH
 UniProt Gene Name    AASS [Similar Products]
 UniProt Synonym Gene Names    LKR; LOR; SDH [Similar Products]
 UniProt Entry Name    AASS_HUMAN
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 NCBI Summary for AASS    This gene encodes a bifunctional enzyme that catalyzes the first two steps in the mammalian lysine degradation pathway. The N-terminal and the C-terminal portions of this enzyme contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively, resulting in the conversion of lysine to alpha-aminoadipic semialdehyde. Mutations in this gene are associated with familial hyperlysinemia. [provided by RefSeq, Jul 2008]
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 UniProt Comments for AASS    AASS: Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively. Defects in AASS are the cause of hyperlysinemia (HYPLYS). Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria.

Protein type: EC 1.5.1.8; Oxidoreductase; EC 1.5.1.9; Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - lysine biosynthesis; Mitochondrial

Chromosomal Location of Human Ortholog: 7q31.3

Cellular Component: intracellular membrane-bound organelle; mitochondrion; mitochondrial matrix

Molecular Function: saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity; saccharopine dehydrogenase (NADP+, L-lysine-forming) activity

Biological Process: L-lysine catabolic process to acetyl-CoA via saccharopine; lysine catabolic process; protein tetramerization

Disease: Saccharopinuria; Hyperlysinemia, Type I
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 Research Articles on AASS    1. Observational study of gene-disease association. (HuGE Navigator)
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-AASS antibodyPathways associated with anti-AASS antibody
 Reference Product  PubMed Publications
 SRM antibody  >1 publications with AASS and SRM
 Products by Pathway  Pathway Diagram
 Lysine Catabolism Pathway antibodies  Lysine Catabolism Pathway Diagram
 Lysine Degradation Pathway antibodies  Lysine Degradation Pathway Diagram
 Lysine Degradation Pathway antibodies  Lysine Degradation Pathway Diagram
 Lysine Degradation, Lysine => Saccharopine => Acetoacetyl-CoA Pathway antibodies  Lysine Degradation, Lysine => Saccharopine => Acetoacetyl-CoA Pathway Diagram
 Lysine Degradation, Lysine => Saccharopine => Acetoacetyl-CoA Pathway antibodies  Lysine Degradation, Lysine => Saccharopine => Acetoacetyl-CoA Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram
 Lysine Degradation II Pathway antibodies  Lysine Degradation II Pathway Diagram
 Lysine Degradation II Pathway antibodies  Lysine Degradation II Pathway Diagram
Diseases associated with anti-AASS antibodyOrgans/Tissues associated with anti-AASS antibody
 Disease Name  Pubmed Publications
 Cardiovascular Diseases Antibodies  >51 publications with AASS and Cardiovascular Diseases
 Heart Diseases Antibodies  >36 publications with AASS and Heart Diseases
 Nervous System Diseases Antibodies  >30 publications with AASS and Nervous System Diseases
 Neoplasm Metastasis Antibodies  >12 publications with AASS and Neoplasm Metastasis
 Necrosis Antibodies  >11 publications with AASS and Necrosis
 Inflammation Antibodies  >9 publications with AASS and Inflammation
 Lung Diseases Antibodies  >8 publications with AASS and Lung Diseases
 Hypertrophy Antibodies  >7 publications with AASS and Hypertrophy
 Immune System Diseases Antibodies  >6 publications with AASS and Immune System Diseases
 Liver Neoplasms Antibodies  >5 publications with AASS and Liver Neoplasms
 Organ/Tissue Name  Pubmed Publications
 Heart Antibodies  >62 publications with AASS and Heart
 Blood Antibodies  >58 publications with AASS and Blood
 Muscle Antibodies  >49 publications with AASS and Muscle
 Bone Antibodies  >29 publications with AASS and Bone
 Brain Antibodies  >27 publications with AASS and Brain
 Liver Antibodies  >17 publications with AASS and Liver
 Lymph Node Antibodies  >16 publications with AASS and Lymph Node
 Testis Antibodies  >13 publications with AASS and Testis
 Lung Antibodies  >13 publications with AASS and Lung
 Nerve Antibodies  >12 publications with AASS and Nerve
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