Niemann protein

Atmann, S.W. et al., Niemann-Rick Cl Like 1 protein is critical for intestinal cholesterol absorption, Science, 303, 1201, 2004. 

Niemann-Pick disease C type 2 Gmj activator protein deficiency Sphingolipid activator protein deficiency... 

In the very rare and fatal Niemann-Pick Cl disease lysosomes in cells of the central nervous system and the viscera accumulate LDL-derived cholesterol. Study of the DNA of patients led to discovery of a 1278-residue integral membrane protein, which may be required for the Golgi-mediated transport of unest-erified cholesterol from lysosomes to the ER.189/232 234c... 

Field, F.J., Bom, E., and Mathur, S.N. 2004. Stanol esters decrease plasma cholesterol independently of intestinal ABC sterol transporters and Niemann-Pick Cl-like 1 protein gene expression. J. Lipid Res. 45, 2252-2259. 

Niemann H (1991) Molecular biology of clostridial neurotoxins. In Alouf J, Freer J (eds) A source-book of bacterial protein toxins. Academic Press, London, pp 303 18 Nishiki T, Tokuyama Y, Kamata Y, Nemoto Y, Yoshida A et al. (1996) The high-affinity binding of Clostridium botulinum type b neurotoxin to synaptotagmin ii associated with gangliosides gtlb/gdla. FEBS Lett 378 253-7... 

Cholesterol is absorbed by intestinal epithelial cells via a Niemann-Pick Cl-Like 1 (NPCILI) protein. This is the target of a number of anti-hyperhpidaemic drugs used to lower cholesterol levels. Examples include miglustafi aUopregnanolone, oxysterols and cyclodextrins all are able to slow the progress of the disease, but none as yet provides an... 

To put an end to what he saw as a distraction from more valuable lines of inquiry, Pauling and fellow protein researcher Carl Niemann in 1939 published a paper marshaling all the chemical evidence in favor of their chain theory of protein structure and against Wrinch s arguments. Its impact was devastating. After it appeared, no one would take Wrinch s cyclols seriously again. 

Blast, J., Chapman, E.R., Link, E., Binz, T., Yamasaki, S., DeC-amilli, P., Sudoff, T.C., Niemann, H., Jahn, R. (1993). Botulinum neurotoxin A selectively cleaves the synaptic protein SNAP-25. Nature 365 160-3. 

Fischer (1906) recognized the possibility of linkages between active side chains. Pauling and Niemann (1939) suggested that covalent cross-links such as disulfide bridges, side-chain ester links, and side-chain peptide links might be partly responsible for the configurational stability of proteins. 

Black JD, Dolly JO (1986 b) Interaction of l-labeled neurotoxins with nerve terminals. II. Autoradiographic evidence for its uptake into motor nerves by acceptor-mediated endocytosis. In J. Cell Biol. 103 535-44 Blasi J, Chapman ER, Link E, Binz T, Yamasaki S, DeCamilli P, SudhofTC, Niemann H, Jahn R (1993) Botulinum neurotoxin A selectively cleaves the synaptic protein SNAP-25. In Nature 365 160-3... 

Tetanus toxin (TeTx) is a zinc-dependent protease that has been shown to cleave VAMP-1, VAMP-2 and cellubrevin (Niemann ef al., 1994). The toxin usually cleaves between a glutamine and a phenylalanine residue. The cleavage site of rat VAMP-1 is slightly different as the glutamine residue is replaced by valine. For this reason rat VAMP-1 is a poor substrate for TeTx (Niemann et al., 1994). Since the toxin is highly specific for VAMPs, it is a powerful tool to investigate the role of these proteins in different cellular processes. 

Altmann SW, Davis HR Jr, Zhu LJ, Yao X, Hoos LM, Tetzloff G, Iyer SP, Maguire M, Golovko A, Zeng M, Wang L, Murgolo N, Graziano MP (2004) Niemann-Pick Cl Like 1 protein is critical for intestinal cholesterol absorption. Science 303 1201-1204... 

Choudhury A, Dominguez M, Puri V, et al. (2002) Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells. 1 Clin Invest 109 1541-1550... 

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