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Rabbit Anti-Lamin B2/FITC Conjugated antibody
background:
A unique family of Cysteine proteases has been described that differs in sequence, structure and substrate specificity from any previously described protease family. This family, termed CED-3/ICE, functions as key components of the apoptotic machinery and act to destroy specific target proteins which are critical to cellular longevity. Nuclear lamins are critical to maintaining the integrity of the nuclear envelope and cellular morphology as components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin. B-type lamins undergo a series of modifications, such as farnesylation and phosphorylation. Increased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations. Nuclear Lamin B is fragmented as a consequence of apoptosis by an unidentified member of the ICE family.
Function:
Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin.
Subunit:
Interacts with TMEM43 (By similarity).
Subcellular Location:
Nucleus inner membrane; Lipid-anchor; Nucleoplasmic side.
Post-translational modifications:
B-type lamins undergo a series of modifications, such as farnesylation and phosphorylation. Increased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations.
DISEASE:
Defects in LMNB2 are a cause of partial acquired lipodystrophy (APLD) [MIM:608709]. A rare childhood disease characterized by loss of subcutaneous fat from the face and trunk. Fat deposition on the pelvic girdle and lower limbs is normal or excessive. Most frequently, onset between 5 and 15 years of age. Most affected subjects are females and some show no other abnormality, but many develop glomerulonephritis, diabetes mellitus, hyperlipidemia, and complement deficiency. Mental retardation in some cases. APLD is a sporadic disorder of unknown etiology.
Similarity:
Belongs to the intermediate filament family.
Database links:
Entrez Gene: 84823 Human
Entrez Gene: 16907 Mouse
Entrez Gene: 299625 Rat
Omim: 150341 Human
SwissProt: Q03252 Human
SwissProt: P21619 Mouse
Unigene: 728836 Human
Unigene: 7362 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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