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Mouse Anti-GLRX5 Recombinant Antibody (4G8) (CBMAB-G3884-LY)

This product is antibody recognizes GLRX5. The antibody 4G8 immunoassay techniques such as: ELISA, WB.
See all GLRX5 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
4G8
Antibody Isotype
IgG2b, κ
Application
ELISA, WB

Basic Information

Immunogen
GLRX5 (NP_057501.2, 1 a.a. ~ 157 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.Immunogen sequence: MSGSLGRAAA ALLRWGRGAG GGGLWGPGVR AAGSGAGGGG SAEQLDALVK KDKVVVFLKG TPEQPQCGFS NAVVQILRLH GVRDYAAYNV LDDPELRQGI KDYSNWPTIP QVYLNGEFVG GCDILLQMHQ NGDLVEELKK LGIHSALLDE KKDQDSK
Specificity
Human
Antibody Isotype
IgG2b, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
glutaredoxin 5
Introduction
This gene encodes a mitochondrial protein, which is evolutionarily conserved. It is involved in the biogenesis of iron-sulfur clusters, which are required for normal iron homeostasis. Mutations in this gene are associated with autosomal recessive pyridoxine-refractory sideroblastic anemia. [provided by RefSeq, May 2010]
Entrez Gene ID
51218
UniProt ID
Q86SX6
Alternative Names
Glutaredoxin 5; Monothiol Glutaredoxin-5; C14orf87; Glutaredoxin-Related Protein 5; Mitochondrial; Glutaredoxin 5 Homolog (S. Cerevisiae); Chromosome 14 Open Reading Frame 87; Glutaredoxin 5 Homolog; FLB4739;
Function
Monothiol glutaredoxin involved in mitochondrial iron-sulfur (Fe/S) cluster transfer (PubMed:20364084, PubMed:23615440).

Receives 2Fe/2S clusters from scaffold protein ISCU and mediates their transfer to apoproteins, to the 4Fe/FS cluster biosynthesis machinery, or export from mitochondrion (PubMed:20364084, PubMed:23615440, PubMed:24334290).

Required for normal regulation of hemoglobin synthesis by the iron-sulfur protein ACO1 (PubMed:20364084).
Biological Process
[2Fe-2S] cluster assembly Source: UniProtKB
Hemopoiesis Source: UniProtKB
Protein maturation by [2Fe-2S] cluster transfer Source: UniProtKB
Protein maturation by [4Fe-4S] cluster transfer Source: UniProtKB
Cellular Location
Mitochondrion matrix
Involvement in disease
Anemia, sideroblastic, 3, pyridoxine-refractory (SIDBA3):
A form of sideroblastic anemia, a bone marrow disorder defined by the presence of pathologic iron deposits in erythroblast mitochondria. Sideroblastic anemia is characterized by anemia of varying severity, hypochromic peripheral erythrocytes, systemic iron overload secondary to chronic ineffective erythropoiesis, and the presence of bone marrow ringed sideroblasts. Sideroblasts are characterized by iron-loaded mitochondria clustered around the nucleus. SIDBA3 is refractory to treatment with vitamin B6, while iron chelation therapy may result in clinical improvement. SIDBA3 inheritance is autosomal recessive.
Spasticity, childhood-onset, with hyperglycinemia (SPAHGC):
An autosomal recessive disorder characterized by childhood-onset of spasticity, spinal lesions, leukodystrophy, optic atrophy in some patients, non-ketotic hyperglycinemia, and defective enzymatic glycine cleavage. Glycine levels in the cerebrospinal fluid are mildly increased in some but not all patients. The increase is less pronounced than in patients with classic non-ketotic hyperglycinemia.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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