Rabbit anti-Human ALDH6A1 Polyclonal Antibody | anti-ALDH6A1 antibody

ALDH6A1, CT (ALDH6A1, MMSDH, Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial, Aldehyde dehydrogenase family 6 member A1) (FITC)

Cat. Num. AAA6272820

• Gene Names: ALDH6A1; MMSDH; MMSADHA
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ALDH6A1; Polyclonal Antibody; CT (ALDH6A1; MMSDH; Methylmalonate-semialdehyde dehydrogenase [acylating]; mitochondrial; Aldehyde dehydrogenase family 6 member A1) (FITC); Aldehyde dehydrogenase family 6 member A1; anti-ALDH6A1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-ALDH6A1 antibody: Immunohistochemistry (IHC), FLISA, Western Blot (WB)
• Application Notes: IHC: 1:10-50; FLISA: 1:1,000; WB: 1:50-100; Applications are based on unconjugated antibody.
• Immunogen: ALDH6A1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 374~404 amino acids from the C-terminal region of human ALDH6A1.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• 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. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human hepatocarcinoma tissue reacted with *ALDH6A1 antibody (C-term), 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.)

Western Blot (WB)

(Western blot analysis of ALDH6A1 (arrow) using rabbit polyclonal ALDH6A1 Antibody (C-term) (RB11957). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the ALDH6A1 gene (Lane 2) (Origene Technologies).)

Related Product Information for anti-ALDH6A1 antibody

ALDH6A1 belongs to the aldehyde dehydrogenases family of proteins. This enzyme plays a role in the valine and pyrimidine catabolic pathways. This protein is a mitochondrial methylmalonate semialdehyde dehydrogenase, and 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.

Product Categories/Family for anti-ALDH6A1 antibody

Antibodies; Mitochondrial Proteins

References

Kuiper, H., Cytogenet. Genome Res. 109 (4), 533 (2005). Anderson, N.L., Mol. Cell Proteomics 3 (4), 311-326 (2004). Chambliss, K.L., J. Inherit. Metab. Dis. 23 (5), 497-504 (2000). Kedishvili, N.Y., J. Biol. Chem. 267 (27), 19724-19729 (1992).

NCBI and Uniprot Product Information

• NCBI GI #: 12643424
• NCBI GeneID: 4329
• UniProt Accession #: Q02252; Q9UKM8; B2R609; B4DFS8; J3KNU8
• Molecular Weight: 535
• NCBI Official Full Name: Methylmalonate-semialdehyde dehydrogenase
• NCBI Official Synonym Full Names: aldehyde dehydrogenase 6 family, member A1
• NCBI Official Symbol: ALDH6A1
• NCBI Official Synonym Symbols: MMSDH; MMSADHA
• NCBI Protein Information: methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial; mitochondrial acylating methylmalonate-semialdehyde dehydrogenase
• UniProt Protein Name: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
• UniProt Gene Name: ALDH6A1
• UniProt Synonym Gene Names: MMSDH; MMSDH; Malonate-semialdehyde dehydrogenase [acylating]
• UniProt Entry Name: MMSA_HUMAN

NCBI Description

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]

Uniprot Description

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: Carbohydrate Metabolism - inositol phosphate; EC 1.2.1.27; EC 1.2.1.18; Mitochondrial; Oxidoreductase; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation

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; thymine catabolic process; valine metabolic process; branched chain family amino acid catabolic process

Disease: Methylmalonate Semialdehyde Dehydrogenase Deficiency