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APOLIPOPROTEIN B

APOLIPOPROTEIN B

Hersteller	GENWAY
Typ	Proteins
Specific against	other
Applikationen	ELISA
Menge	0,5 mg
ArtNr	11-783-79481
eClass 6.1	34160400
eClass 9.0	42020190
Lieferbar
Genway ID:	GWB-4274C2
Specificity:	APOLIPOPROTEIN B
Preparation:	Ultracentrifugation in density gradient. Delipidation with diethyl ether/ethanol. Gel filtration on Sephacryl S-300.
Buffer Solution:	50 mM Na2CO3 pH 10 50 mM NaCl and deoxycholate
Function:	Apolipoprotein B is a major protein constituent of chylomicrons (apo B-48) LDL (apo B-100) and VLDL (apo B-100). Apo B-100 functions as a recognition signal for the cellular binding and internalization of LDL particles by the apoB/E receptor.
Subcellular Location:	Secreted.
Ptm:	Palmitoylated; structural requirement for proper assembly of the hydrophobic core of the lipoprotein particle. Rna Editing: Modified_positions=2180; Note=The stop codon (UAA) at position 2180 is created by RNA editing. Apo B-48 derived from the fully edited RNA is produced only in the intestine and is found in chylomicrons. Apo B-48 is a shortened form of apo B-100 which lacks the LDL-receptor region. The unedited version (apo B-100) is produced by the liver and is found in the VLDL and LDL.
Disease:	Defects in APOB are a cause of familial hypobetalipoproteinemia (FHBL) [MIM:107730]. FHBL is a genetically heterogeneous autosomal co-dominant disorder associated with reduced plasma concentrations of apoB LDL and VLDL. Heterozygotes for FHBL are usually asymptomatic with LDL cholesterol and apoB-100 concentrations less than 50% of those in normal plasma. Homozygotes have extremely low plasma LDL cholesterol and apoB-100 concentrations and clinical presentation may vary from no symptoms to severe gastrointestinal and neurological dysfunction similar to abetalipoproteinemia [MIM:200100].
Disease:	Defects in APOB are a cause of familial ligand-defective apolipoprotein B-100 (FDB) [MIM:144010]. FDB is a dominantly inherited disorder of lipoprotein metabolism leading to hypercholesterolemia and increased proneness to coronary artery disease (CAD). The plasma cholesterol levels are dramatically elevated due to impaired clearance of LDL particles by defective APOB/E receptors.
Disease:	Defects in APOB associated with defects in other genes (polygenic) can contribute to hypocholesterolemia.
Similarity:	Contains 1 vitellogenin domain. 1. Laemmli. U. (1970) Cleavage of structural proteins during the assembly of the head of bacteriophage T4 2. Ginsburg. G. S. et al. (1984) Reassembled plasma low density lipoproteins. Phospholipid-cholesterol ester-apoprotein B complexes. 3. Helenius. A. & Simons. K. (1971) Removal of lipids from human plasma low-density lipoprotein by detergents.

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Menge: 0,5 mg

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