EIF2B2 Antibody
Description
Introduction
The EIF2B2 antibody is a laboratory tool designed to detect and study the eIF2B2 protein, a critical subunit of the eukaryotic translation initiation factor 2B (eIF2B) complex. This complex regulates global protein synthesis by facilitating the exchange of GDP for GTP on the eIF2 protein, enabling the initiation of translation . Mutations in the EIF2B2 gene are linked to severe neurological disorders, such as leukoencephalopathy with vanishing white matter (VWM) , making this antibody vital for researching disease mechanisms and therapeutic interventions.
Applications of EIF2B2 Antibody
The antibody is widely used in molecular biology techniques to investigate eIF2B2 expression, localization, and interactions:
Techniques
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Western Blot (WB): Detects denatured eIF2B2 in cell lysates or tissue extracts .
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Immunohistochemistry (IHC): Stains eIF2B2 in paraffin-embedded or frozen tissue sections, aiding histopathological analysis .
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Immunofluorescence (IF/ICC): Visualizes eIF2B2 in fixed cells for subcellular localization studies .
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Immunoprecipitation (IP): Captures eIF2B2 complexes for downstream analysis of protein interactions .
Role in Vanishing White Matter Disease (VWM)
Mutations in EIF2B2 impair eIF2B activity, leading to chronic stress responses and neurodegeneration . Studies using EIF2B2 antibodies have demonstrated:
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Reduced eIF2B complex stability and GEF activity in mutant cells .
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Therapeutic potential of eIF2B activators (e.g., 2BAct), which stabilize mutant complexes and restore normal translation .
Stress Response and Translation Regulation
eIF2B2 antibodies have been instrumental in studying how phosphorylated eIF2α inhibits eIF2B activity during cellular stress (e.g., amino acid deprivation, viral infection) . For example:
Product Specs
Target Background
- Data show that eIF2Balpha and eIF2Bbeta bind to adjacent surfaces on eIF2alpha-N-terminal domains (NTDs). PMID: 29036434
- It is more accurate to consider Vanishing White Matter Disease as an eIF2B-related multisystem disorder, rather than solely a neurological disorder. PMID: 28041799
- A case study reports an Italian patient with a c.638A>G mutation in exon 5 of the EIF2B2 gene, exhibiting very slow progressive vanishing white matter disease. PMID: 22729508
- An analysis of vanishing white matter disease caused by EIF2B2 mutation presents a case report of adrenoleukodystrophy phenotype. PMID: 22285377
- Mutations in EIF2B2 are associated with childhood ataxia, central nervous system hypomyelination, and vanishing white matter leukodystrophy. PMID: 12707859
- Biochemical analyses indicate that mutations in eIF2Balpha and -epsilon reduce the steady-state level of the affected subunit. The most severe mutant tested, eIF2Bbeta(V341D), forms complexes with reduced stability and lower eIF2B activity. PMID: 14993275
- The residues Ser2 and Ser67 play a crucial role in the N-terminal region of eIF2beta for its function in mammals. PMID: 16225457
- CACH syndrome is linked to mutations in the five EIF2B genes – a review. PMID: 17878805
- This study reports 9 novel mutations in EIF2B genes in 8 patients, increasing the number of known mutations to over 120. Using homology modeling, the impact of novel mutations on the 5 subunits of the eIF2B protein (alpha, beta, gamma, delta, epsilon) was analyzed. PMID: 18263758
