Immunogen
Synthesized peptide derived from human HEM1 AA range: 87-137
Specificity
This antibody detects endogenous levels of HEM1 at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1:500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
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],
Function
catalytic activity:Succinyl-CoA + glycine = 5-aminolevulinate + CoA + CO(2).,cofactor:Pyridoxal phosphate.,miscellaneous:There are two delta-ALA synthetase in vertebrates: an erythroid- specific form and one (housekeeping) which is expressed in all tissues.,pathway:Porphyrin metabolism; protoporphyrin-IX biosynthesis; 5-aminolevulinate from glycine: step 1/1.,similarity:Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.,subunit:Homodimer.,
Gene Name
ALAS1 ALAS3 ALASH OK/SW-cl.121
Fields
>>Glycine, serine and threonine metabolism;>>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors
Human protein sequence Database
P13196
Mouse protein sequence database
Q8VC19
Rat protein sequence database
P13195
Cellular localization
Mitochondrion inner membrane ; Peripheral membrane protein . Localizes to the matrix side of the mitochondrion inner membrane. .