Mouse Glrx5 ELISA Kit (V2LY-0626-LY5876)
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Basic Information
- Pre-coated ELISA Plate: 12 wells * 8 detachable strips
- Standard solution: 0.5ml x1
- Standard diluent: 3ml x1
- Streptavidin-HRP: 6ml x1
- Stop solution: 6ml x1
- Substrate solution A: 6ml x1
- Substrate solution B: 6ml x1
- Wash buffer concentrate (25x): 20ml x1
- Biotinylated antibody: 1ml x1
Formulations & Storage [For reference only, actual COA shall prevail!]
Target
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).
Hemopoiesis Source: UniProtKB
Protein maturation by [2Fe-2S] cluster transfer Source: UniProtKB
Protein maturation by [4Fe-4S] cluster transfer Source: UniProtKB
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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Please try the standard protocols which include: protocols, troubleshooting and guide.
Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols
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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