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AASS Antibody – Rabbit Polyclonal

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS6460807

MBS6460807 is an antibody targeting AASS. Also known as AASS, ID (Alpha-aminoadipic Semialdehyde Synthase, Mitochondrial, LKR/SDH, Lysine Ketoglutarate Reductase, LKR, LOR, Saccharopine Dehydrogenase) (FITC). Reactive in Human, Mouse. Host: Rabbit. More details

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Product Documentation
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Sample Manual Insert View PDF
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Testing Data View PDF
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Certificate of Analysis (COA) View PDF
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QC Report View PDF
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Material Safety Data Sheet View PDF
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Technical Datasheet View PDF
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Protocol View PDF

Product Overview

Online Datasheet in PDF
Product Name
AASS, Polyclonal Antibody
Full Product Name
AASS, ID (Alpha-aminoadipic Semialdehyde Synthase, Mitochondrial, LKR/SDH, Lysine Ketoglutarate Reductase, LKR, LOR, Saccharopine Dehydrogenase) (FITC)
Product Gene Name
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse
Form/Format
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).
Purity/Purification
Purified by Ammonium Sulfate Precipitation
Specificity
Recognizes human AASS. Species Crossreactivity: mouse
Preparation and Storage
Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer.
Molecular Weight
102.001
OMIM
238700
3D Structure
ModBase 3D Structure for Q9UDR5
Immunogen
Synthetic peptide corresponding to a portion of amino acids within aa272-301 from the center region of human AASS. conjugated to KLH.
Conjugate
FITC
Note
Preservative Free

Product Categories/Family for anti-AASS antibody

Applications Tested/Suitable for anti-AASS antibody
Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)
Application Notes for anti-AASS antibody
WB: 1:50-1:100
ELISA: 1:1000
Applications are based on unconjugated antibody.

Western Blot (WB) of anti-AASS antibody

Western blot analysis of AASS Antibody (Center) in 293 cell line lysates (35ug/lane). AASS (arrow) was detected using the purified Pab.
Rabbit Anti-AASS Polyclonal Antibody – Cat# MBS6460807

Immunohistochemistry (IHC) of anti-AASS antibody

Formalin-fixed and paraffin-embedded human hepatocarcinoma tissue reacted with the AASS antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.
Rabbit Anti-AASS Polyclonal Antibody, Immunohistochemistry (IHC) – Cat# MBS6460807

Western Blot (WB) of anti-AASS antibody

AASS Antibody (Center) western blot analysis in mouse liver tissue lysates (35ug/lane).This demonstrates the AASS antibody detected the AASS protein (arrow).
Rabbit Anti-AASS Polyclonal Antibody, Western Blot (WB) – Cat# MBS6460807

Product References and Citations for anti-AASS antibody

1. Sacksteder, K.A., Am. J. Hum. Genet. 66(6), 1736-1743 (2000).

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.

Additional Details

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 #
UniProt Primary Accession #
UniProt Related Accession #
GenBank Nucleotide #
NCBI Official Full Name
alpha-aminoadipic semialdehyde synthase, mitochondrial
NCBI Official Synonym Full Names
aminoadipate-semialdehyde synthase
NCBI Official Symbol
AASS
NCBI Official Synonym Symbols
LKRSDH; LORSDH; LKR/SDH
UniProt Protein Name
Alpha-aminoadipic semialdehyde synthase, mitochondrial
UniProt Synonym Protein Names
LKR/SDH
UniProt Gene Name
AASS
UniProt Synonym Gene Names
LKR; LOR; SDH
UniProt Entry Name
AASS_HUMAN
NCBI Protein Information
alpha-aminoadipic semialdehyde synthase, mitochondrial

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; EC 1.5.1.9; Oxidoreductase; Mitochondrial; Amino Acid Metabolism - lysine biosynthesis; Amino Acid Metabolism - lysine degradation

Chromosomal Location of Human Ortholog: 7q31.3

Cellular Component: mitochondrion; intracellular membrane-bound organelle; 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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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.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

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