Alad/ALADH/Porphobilinogen synthase

For Research Use Only. Not for use in diagnostic procedures.

Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

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Small volumes of Alad elisa kit 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.

MBS763024 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Aminolevulinate Delta Dehydratase (Alad) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing Alad. The ELISA analytical biochemical technique of the MBS763024 kit is based on Alad antibody-Alad antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect Alad antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, Alad. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. anti-Rat Alad antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Rat Alad antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Rat Alad amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of Rat Alad can be calculated.

39,034 Da

aminolevulinate dehydratase

delta-aminolevulinic acid dehydratase

Delta-aminolevulinic acid dehydratase

ALADH??[Similar Products]

HEM2_HUMAN

The ALAD enzyme is composed of 8 identical subunits and catalyzes the condensation of 2 molecules of delta-aminolevulinate to form porphobilinogen (a precursor of heme, cytochromes and other hemoproteins). ALAD catalyzes the second step in the porphyrin and heme biosynthetic pathway; zinc is essential for enzymatic activity. ALAD enzymatic activity is inhibited by lead and a defect in the ALAD structural gene can cause increased sensitivity to lead poisoning and acute hepatic porphyria. Alternative splicing of this gene results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Dec 2015]

ALAD: Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen. Defects in ALAD are the cause of acute hepatic porphyria (AHEPP). A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Belongs to the ALADH family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 4.2.1.24; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Lyase

Chromosomal Location of Human Ortholog: 9q33.1

Cellular Component: cytosol; extracellular space; nucleus

Molecular Function: catalytic activity; identical protein binding; lead ion binding; porphobilinogen synthase activity; zinc ion binding

Biological Process: heme biosynthetic process; porphyrin metabolic process; protein homooligomerization; protoporphyrinogen IX biosynthetic process; response to activity; response to aluminum ion; response to amino acid stimulus; response to arsenic; response to cadmium ion; response to cobalt ion; response to drug; response to ethanol; response to glucocorticoid stimulus; response to herbicide; response to hypoxia; response to ionizing radiation; response to iron ion; response to lipopolysaccharide; response to mercury ion; response to methylmercury; response to oxidative stress; response to selenium ion; response to vitamin B1; response to vitamin E; response to zinc ion

Disease: Porphyria, Acute Hepatic

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