Disease relevance of Acsbg1

• Western blot analysis of BG1 protein levels showed no differences between fibroblasts from controls, cerebral X-ALD, or adrenomyeloneuropathy patients [1].
• It has been suggested that a gene termed bubblegum (Bgm), encoding an acyl-CoA synthetase, could be involved in the pathogenesis of the inherited neurodegenerative disorder X-ALD (X-linked adrenoleukodystrophy) [2].
• The ether lipid analogue 1-octadecyl-2-methyl-rac-glycero-3-phosphocholine (ET-18-OCH3) has been shown to be a direct inhibitor of Swiss 3T3 fibroblast and BG1 ovarian adenocarcinoma cell cytosolic phosphoinositide selective phospholipase C (PIPLC) using [3H]-phosphatidylinositol-(4, 5)-bisphosphate ([3H]PIP2) as the substrate [3].

High impact information on Acsbg1

• BG1 cells, which were more sensitive than Swiss 3T3 fibroblasts to growth inhibition by ET-18-OCH3, had a cytosolic PIPLC activity one-third that of Swiss 3T3 cells [3].
• These (1-->3,1-->4)-beta-oligoglucosides, together with (1-->3)-beta- and (1-->4)-beta-oligoglucosides were used to characterize the binding site of the monoclonal antibody (BG1) by competitive inhibition [4].
• A novel mammalian bubblegum-related acyl-CoA synthetase restricted to testes and possibly involved in spermatogenesis [5].
• Thus, the BGR gene expands the bubblegum ACS family with a testes-specific, developmentally regulated member that may play a role in spermatogenesis [5].
• Two enzymes with VLACS activity, ACSVL1 and BG1, were investigated for their potential role in X-ALD biochemical pathology [1].

Biological context of Acsbg1

• However, BG1 depletion in Neuro2a cells using RNA interference did not decrease incorporation of labeled VLCFA into cholesterol esters [1].

Anatomical context of Acsbg1

• Similar results were obtained with mRNA for BG1, a non-peroxisomal enzyme that is highly expressed in nervous system, adrenal gland, and testis, the principal tissues pathologically affected in X-ALD [1].
• BG1 protein levels were similar in wild-type and X-ALD mouse brain, spinal cord, testis, and adrenal gland [1].

Associations of Acsbg1 with chemical compounds

• We hypothesized that one function of BG1 was to direct VLCFA into the cholesterol ester synthesis pathway [1].

Other interactions of Acsbg1

• We conclude that the role, if any, of ACSVL1 and BG1 in X-ALD biochemical pathology is indirect [1].
• Murine bubblegum orthologue is a microsomal very long-chain acyl-CoA synthetase [2].

Analytical, diagnostic and therapeutic context of Acsbg1

• The monoclonal antibody (BG1) has been used to develop a sensitive sandwich ELISA for the specific quantitation of (1-->3,1-->4)-beta-glucans [4].

References