ELISA/assay

Human Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial, ALDH6A1 ELISA Kit

MBS9318078

48-Strip-Wells

435 USD

ELISA Kit

Human Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial, ALDH6A1 ELISA Kit

aldehyde dehydrogenase 6 family, member A1

Human Methylmalonate-semialdehyde dehydrogenase [acylating]; mitochondrial (ALDH6A1) ELISA kit; MGC40271; MMSADHA; MMSDH; aldehyde dehydrogenase 6A1; mitochondrial acylating methylmalonate-semialdehyde dehydrogenase; aldehyde dehydrogenase 6 family; member A1

methylmalonate-semialdehyde dehydrogenase; Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; mitochondrial acylating methylmalonate-semialdehyde dehydrogenase; aldehyde dehydrogenase 6 family, member A1; Aldehyde dehydrogenase family 6 member A1

ALDH6A1

ALDH6A1; ALDH6A1; MMSDH; MMSADHA; MMSDH; MMSDH; Malonate-semialdehyde dehydrogenase [acylating]

MMSA_HUMAN

Human

No significant cross-reactivity or interference between Human ALDH6A1 and analogues was observed.

Store all reagents at 2-8 degree C

Samples: Serum, Plasma, Tissue Homogenate, Feces and Urine. Assay Type: Sandwich. Detection Range: 0.25 ng/ml - 8 ng/ml. Sensitivity: 0.1 ng/ml.

Intended Uses: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! It is intended to be determinated ALDH6A1 concentrations in Human serum, plasma and other body fluids. Using Purified Human ALDH6A1 antibody to coat Microelisa Stripplate wells to make solid-phase antibody, then add ALDH6A1 and ALDH6A1 antibody which has been labeled with HRP to wells, then the reactants become antibody-antigen-antibody-enzyme complex, after washing completely, add TMB substrate solution, TMB substrate becomes blue color under HRP enzyme-catalyzed, reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. The concentration of ALDH6A1 in the samples is then determined by comparing the O.D. of the samples to the standard curve. Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100]

Background: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! It is intended to be determinated ALDH6A1 concentrations in Human serum, plasma and other body fluids. Using Purified Human ALDH6A1 antibody to coat Microelisa Stripplate wells to make solid-phase antibody, then add ALDH6A1 and ALDH6A1 antibody which has been labeled with HRP to wells, then the reactants become antibody-antigen-antibody-enzyme complex, after washing completely, add TMB substrate solution, TMB substrate becomes blue color under HRP enzyme-catalyzed, reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. The concentration of ALDH6A1 in the samples is then determined by comparing the O.D. of the samples to the standard curve.

515870065

NP_001265522.1

NM_001278593.1

Q02252

57,840 Da

Branched-chain Amino Acid Catabolism Pathway (106179); Inositol Phosphate Metabolism Pathway (82987); Inositol Phosphate Metabolism Pathway (362); Malonate Semialdehyde Pathway, Propanoyl-CoA = Acetyl-CoA (413478); Malonate Semialdehyde Pathway, Propanoyl-CoA = Acetyl-CoA (468206); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Propanoate Metabolism Pathway (83004); Propanoate Metabolism Pathway (387); Valine, Leucine And Isoleucine Degradation Pathway (82952)

This gene encodes a member of the aldehyde dehydrogenase protein family. The encoded protein is a mitochondrial methylmalonate semialdehyde dehydrogenase that plays a role in the valine and pyrimidine catabolic pathways. This protein catalyzes the irreversible oxidative decarboxylation of malonate and methylmalonate semialdehydes to acetyl- and propionyl-CoA. Methylmalonate semialdehyde dehydrogenase deficiency is characterized by elevated beta-alanine, 3-hydroxypropionic acid, and both isomers of 3-amino and 3-hydroxyisobutyric acids in urine organic acids. Alternate splicing results in multiple transcript variants. [provided by RefSeq, Jun 2013]

ALDH6A1: Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA. Defects in ALDH6A1 are the cause of methylmalonate semialdehyde dehydrogenase deficiency (MMSDHD). This is characterized by elevated beta-alanine, 3-hydroxypropionic acid, and both isomers of 3-amino and 3-hydroxyisobutyric acids in urine organic acids. Belongs to the aldehyde dehydrogenase family. Protein type: Mitochondrial; EC 1.2.1.18; Oxidoreductase; EC 1.2.1.27; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; Carbohydrate Metabolism - inositol phosphate. Chromosomal Location of Human Ortholog: 14q24.3. Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix. Molecular Function: aldehyde dehydrogenase (NAD) activity; acyl-CoA binding; methylmalonate-semialdehyde dehydrogenase (acylating) activity; malonate-semialdehyde dehydrogenase (acetylating) activity. Biological Process: valine catabolic process; thymine metabolic process; brown fat cell differentiation; valine metabolic process; thymine catabolic process; branched chain family amino acid catabolic process. Disease: Methylmalonate Semialdehyde Dehydrogenase Deficiency

48-Strip-Wells

435 USD

96-Strip-Wells

600