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

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Catalog # MBS9214873
Unit / Price
  0.08 mL  /  $150 +1 FREE 8GB USB
  0.4 mL  /  $340 +1 FREE 8GB USB
Product Name

AASS, Polyclonal Antibody

Also Known As

AASS Antibody (C-term)

Product Synonym Names
Alpha-aminoadipic semialdehyde synthase; mitochondrial; LKR/SDH; Lysine ketoglutarate reductase; LKR; LOR; Saccharopine dehydrogenase; SDH; AASS
Antibody/Peptide Pairs
AASS peptide (MBS9218126) is used for blocking the activity of AASS antibody (MBS9214873)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Positions
805-834
Immunogen Sequence Length
926
OMIM
238700
3D Structure
ModBase 3D Structure for Q9UDR5
Clonality
Polyclonal
Isotype
Rabbit Ig
Host
Rabbit
Species Reactivity
Human
Specificity
This AASS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 805-834 amino acids from the C-terminal region of human AASS.
Purity/Purification
Purified Rabbit Polyclonal Antibody (Pab)
Form/Format
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
Concentration
Vial Concentration: 2 (lot specific)
Antigen Type
Synthetic Peptide
Antigen Source
HUMAN
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent 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.
Related Product Information for
anti-AASS antibody
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.
Product Categories/Family for anti-AASS antibody
Applications Tested/Suitable for anti-AASS antibody
Western Blot (WB), ELISA (EIA), Flow Cytometry (FC/FACS)
Application Notes for anti-AASS antibody
WB~~1:1000

Western Blot (WB) of anti-AASS antibody
AASS Antibody (C-term) western blot analysis in K562 cell line lysates (35ug/lane).This demonstrates the AASS antibody detected the AASS protein (arrow).
anti-AASS antibody Western Blot (WB) (WB) image
Flow Cytometry (FC/FACS) of anti-AASS antibody
AASS Antibody (C-term) flow cytometric analysis of K562 cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.
anti-AASS antibody Flow Cytometry (FC/FACS) (FC/FACS) image
NCBI/Uniprot data below describe general gene information for AASS. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
102132
NCBI Official Full Name
alpha-aminoadipic semialdehyde synthase, mitochondrial
NCBI Official Synonym Full Names
aminoadipate-semialdehyde synthase
NCBI Official Symbol
NCBI Official Synonym Symbols
LKRSDH; LORSDH; LKR/SDH
  [Similar Products]
NCBI Protein Information
alpha-aminoadipic semialdehyde synthase, mitochondrial
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; LORSaccharopine dehydrogenase (EC:1.5.1.9); SDH
UniProt Gene Name
UniProt Synonym Gene Names
LKR; LOR; SDH  [Similar Products]
UniProt Entry Name
AASS_HUMAN
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]
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; Amino Acid Metabolism - lysine degradation; Mitochondrial; Amino Acid Metabolism - lysine biosynthesis; EC 1.5.1.9

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
Product References and Citations for anti-AASS antibody
Sacksteder, K.A., et al. Am. J. Hum. Genet. 66(6):1736-1743(2000)
Papes, F., et al. Biochem. J. 344 PT 2, 555-563 (1999) :

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.

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Products associated withanti-AASS antibody
 Reference Product  PubMed Publications
 SRM antibody  >1 publications with AASS and SRM
Pathways associated with anti-AASS antibody
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