lamin b การใช้

• The phosphorylated lamin B dimers remain associated with the nuclear membrane via their isoprenyl anchor.
• The major types are LI and LII, which are considered homologs of lamin B 1 and B2.
• Most lamin B mutations appear to be lethal with mutations in lamin B1 causing death at birth in mice.
• It may mediate interaction between chromatin and lamin B . Mutations of this gene has been associated with autosomal recessive HEM / Greenberg skeletal dysplasia.
• The most important are lamin associated polypeptide 1 and 2 ( LAP 1, LAP2 ), emerin, lamin B-receptor ( LBR ), otefin and MAN 1.
• Defects in the genes encoding for nuclear lamin ( such as lamin A and lamin B 1 ) have been implicated in a variety of diseases ( laminopathies ) such as:
• In further stages, the expression pattern of lamin B 1 decreases and there is a gradual increase in the expression of lamin A . Mammalian development seems to progress in a similar way.
• Nuclear envelope proteins that are bound to the lamina include nesprin, emerin, lamina-associated proteins 1 and 2 ( LAP1 and LAP2 ), the lamin B receptor ( LBR ) and MAN1.
• Mutations implicated in other nuclear envelopathies were found in genes coding for lamin-binding proteins such as lamin B receptor ( LBR gene ), emerin ( EMD gene ) and LEM domain-containing protein 3 ( LEMD3 gene ) and prelamin-processing enzymes such as the zinc metalloproteinase STE24 ( ZMPSTE24 gene ).