Rabbit ALAS1 Polyclonal Antibody | anti-ALAS1 antibody

ALAS1 Polyclonal Antibody

Cat. Num. AAA3007832

• Gene Names: ALAS1; ALAS; MIG4; ALAS3; ALASH; ALAS-H
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunofluorescence
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: ALAS1; Polyclonal Antibody; ALAS1 Polyclonal Antibody; 5-aminolevulinate synthase; nonspecific; mitochondrial; ALAS-H; 5-aminolevulinic acid synthase 1; Delta-ALA synthase 1; Delta-aminolevulinate synthase 1; ALAS3; ALASH; OK/SW-cl.121; anti-ALAS1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: ALAS1 polyclonal antibody detects endogenous levels of ALAS1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 640

• Applicable Applications for anti-ALAS1 antibody: Western Blot (WB), Immunofluorescence (IF)
• Application Notes: WB: 1:500-1:2000; IF: 1:50-1:200
• Immunogen: Recombinant full length Human ALAS1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Testing Data

Related Product Information for anti-ALAS1 antibody

5-aminolevulinate synthase 1 (ALAS-H) and 2 (ALAS-E) are two isoforms of ALAS, an enzyme catalyzing the first step of the heme biosynthetic pathway in mammals. The erythroid-specific isoenzyme, ALAS-E, regulates the first step of hematopoietic cell differentation and iron metabolism in the liver. ALAS-H is a housekeeping protein which mediates synthesis of early heme in the mitochondria of most cells. Succinyl CoA associates with ALAS-E in protein conformation change and translocation of ALAS-E into the mitochondria and does not interact with ALAS-H. The ALAS-E 5'-flanking region contains binding sites for nuclear activators such as GATA-1, NF-E2 and EKLF. Since the ALAS gene maps to the X chromosome, mutation of the gene leads to the pyridoxine-refractory X-linked sideroblastic anemia.

NCBI and Uniprot Product Information

• NCBI GI #: 4502025
• NCBI GeneID: 211
• NCBI Accession #: NP_000679.1
• NCBI GenBank Nucleotide #: NP_000679.1
• UniProt Accession #: P13196
• NCBI Official Full Name: 5-aminolevulinate synthase, nonspecific, mitochondrial isoform 1
• NCBI Official Synonym Full Names: 5'-aminolevulinate synthase 1
• NCBI Official Symbol: ALAS1
• NCBI Official Synonym Symbols: ALAS; MIG4; ALAS3; ALASH; ALAS-H
• NCBI Protein Information: 5-aminolevulinate synthase, nonspecific, mitochondrial
• UniProt Protein Name: 5-aminolevulinate synthase, nonspecific, mitochondrial
• Protein Family: Alanine--tRNA ligase
• UniProt Gene Name: ALAS1
• UniProt Synonym Gene Names: ALAS3; ALASH; ALAS-H
• UniProt Entry Name: HEM1_HUMAN

NCBI Description

This gene encodes the mitochondrial enzyme which is catalyzes the rate-limiting step in heme (iron-protoporphyrin) biosynthesis. The enzyme encoded by this gene is the housekeeping enzyme; a separate gene encodes a form of the enzyme that is specific for erythroid tissue. The level of the mature encoded protein is regulated by heme: high levels of heme down-regulate the mature enzyme in mitochondria while low heme levels up-regulate. A pseudogene of this gene is located on chromosome 12. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jan 2015]

Uniprot Description

ALAS1: the mitochondrial enzyme which is catalyzes the rate-limiting step in heme (iron-protoporphyrin) biosynthesis. The enzyme encoded by this gene is the housekeeping enzyme; a separate gene encodes a form of the enzyme that is specific for erythroid tissue. The level of the mature encoded protein is regulated by heme: high levels of heme down-regulate the mature enzyme in mitochondria while low heme levels up-regulate. A pseudogene of this gene is located on chromosome 12. Multiple alternatively spliced variants, encoding the same protein, have been identified. [provided by RefSeq, Dec 2011]

Protein type: Transferase; Amino Acid Metabolism - glycine, serine and threonine; Mitochondrial; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 2.3.1.37

Chromosomal Location of Human Ortholog: 3p21.1

Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; cytoplasm

Molecular Function: 5-aminolevulinate synthase activity; pyridoxal phosphate binding

Biological Process: mitochondrion organization and biogenesis; organelle organization and biogenesis; porphyrin metabolic process; cellular lipid metabolic process; protoporphyrinogen IX biosynthetic process; heme biosynthetic process