BCL2 associated X (BAX) is traditionally thoμght to be regulated by anti-apoptotic BCL-2 family members. BCL-2-associated X protein (BAX) is a critical apoptotic regulator that can be transformed from a cytosolic monomer into a lethal mitochondrial oligomer, The pro-apoptotic BCL-2 protein BAX commits human cells to apoptosis by permeabilizing the outer mitochondrial membrane. BAX activation has been sμggested to require the separation of helix alpha5 from alpha6 - the 'latch' from the 'core' domain - among other conformational changes. BCL-2-associated X (BAX) protein acts as a gatekeeper in regulating mitochondria-dependent apoptosis. Under cellular stress, BAX becomes activated and transforms into a lethal oligomer that causes mitochondrial outer membrane permeabilization (MOMP).
BBOX1, also known as gamma-BBH, belongs to thegamma-BBH/TMLD family. It is highly expressed in kidney and moderately expressed in liver. BBOX1 catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthetic pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. BBOX1 is an inhibition target for mildronate which can be used to treatanginaandmyocardial infarction. Mildronate may also be beneficial for the treatment ofneurological disorder,diabetes, andseizures andalcohol intoxication.
The Lutheran (Lu) blood group and basal cell adhesion molecule (BCAM) antigens are both carried by 2 glycoprotein isoforms of the immunoglobulin superfamily representing receptors for the laminin alpha(5) chain. It is a transmembrane receptor with five immunoglobulin-like domains in its extracellular region, and is therefore classified as a member of the immunoglobulin (Ig) gene family. In addition to red blood cells, Lu/BCAM proteins are expressed in endothelial cells of vascular capillaries and in epithelial cells of several tissues. BCAM/LU has a wide tissue distribution with a predominant expression in the basal layer of the epithelium and the endothelium of blood vessel walls. As designated as CD239 recently, BCAM and LU share a significant sequence similarity with the CD146 (MUC18) and CD166, and themselves are adhesion molecules that bind laminin with high affinity. Laminins are found in all basement membranes and are involved in cell differentiation, adhesion, migration, and proliferation. BCAM is upregulated following malignant transformation of some cell types in vivo and in vitro, thus being a candidate molecule involved in tumor progression. In addition, BCAM interacts with integrin in sickle red cells, and thus may potentially play a role in vaso-occlusive episodes.
The Lutheran (Lu) blood group and basal cell adhesion molecule (BCAM) antigens are both carried by 2 glycoprotein isoforms of the immunoglobulin superfamily representing receptors for the laminin alpha(5) chain. It is a transmembrane receptor with five immunoglobulin-like domains in its extracellular region , and is therefore classified as a member of the immunoglobulin (Ig) gene family. In addition to red blood cells , Lu/BCAM proteins are expressed in endothelial cells of vascular capillaries and in epithelial cells of several tissues. BCAM/LU has a wide tissue distribution with a predominant expression in the basal layer of the epithelium and the endothelium of blood vessel walls. As designated as CD239 recently , BCAM and LU share a significant sequence similarity with the CD146 (MUC18) and CD166 , and themselves are adhesion molecules that bind laminin with high affinity. Laminins are found in all basement membranes and are involved in cell differentiation , adhesion , migration , and proliferation. BCAM is upregulated following malignant transformation of some cell types in vivo and in vitro , thus being a candidate molecule involved in tumor progression. In addition , BCAM interacts with integrin in sickle red cells , and thus may potentially play a role in vaso-occlusive episodes.
Basal cell adhesion molecule (BCAM, CD239) is an immunoglobulin superfamily protein that arises from alternate splicing of the Lutheran blood group molecule (Lu). The ECD of human BCAM contains two Ig-like V-type domains and three Ig-like C2-type domains. It shares 73% aa sequence identity with the ECDs of mouse and rat BCAM. BCAM is widely expressed in epithelial and endothelial tissues including in the vasculature, kidney glomerulus, small intestine, colon, hair follicle outer root sheath, and basal keratinocytes of the skin during inflammation. BCAM is also expressed on vascular and visceral smooth muscle cells and at the neuromuscular junction of skeletal muscle. BCAM is upregulated on carcinomas, particularly ovarian, sarcomas, astrocytomas, and melanomas. It may mediate intracellular signaling. It cooperates with Integrins β1 and αVβ3 as an adhesion receptor for Laminins which contain the α5 chain. The Lutheran isoform is aberrantly phosphorylated in erythroid disorders and can enhance Lamininmediated adhesion of erythrocytes to vascular endothelial cells.
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