Home > Product > Antibody > Rabbit Anti-ALAS1/FITC Conjugated antibody
mitochondrial; nonspecific; 5 aminolevulinate synthase; 5 aminolevulinate synthase nonspecific mitochondrial; 5 aminolevulinic acid synthase; 5-aminolevulinate synthase; 5-aminolevulinic acid synthase 1; Alas 1; ALAS 3; ALAS; ALAS H; ALAS N; ALAS-H; alaS1
Cat:
SL9527R-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Dog,Horse,Rabbit,)
Immunogen:
KLH conjugated synthetic peptide derived from human ALAS1/ALAS-H
Format:
Lyophilized or Liquid
Storage instructions:
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
65kDa
More
Unit:
Price: $
Product PDFs
Datasheet:


background:
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.

Subunit:
Homodimer.

Subcellular Location:
Mitochondrion matrix

Similarity:
Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.

Database links:

Entrez Gene: 211 Human

Entrez Gene: 11655 Mouse

Entrez Gene: 65155 Rat

Omim: 125290 Human

SwissProt: P13196 Human

SwissProt: Q8VC19 Mouse

SwissProt: P13195 Rat

Unigene: 476308 Human

Unigene: 290578 Mouse

Unigene: 97126 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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