Sentences with phrase «huntingtin fragments»

Huntington's disease is caused by an expansion of glutamine residues in the huntingtin protein, altering its function and ultimately resulting in toxic aggregation of huntingtin fragments in neurons.

Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complexes II and III.

Not exact matches

In a nonhuman primate model geneticist Anthony Chan DVM, PhD, and his colleagues at Yerkes developed, rhesus macaques carry a gene encoding a fragment of mutant human huntingtin.

When they stained brain samples taken from human Huntington's victims, they found that the characteristic globs consist of full - length mutant huntingtin, rather than peptide fragments from the mutant.

Things go awry when these fragments are drawn toward the globs of abnormal huntingtin, instead of being processed further.

These three monkeys carried a gene encoding a fragment of mutant human huntingtin.

Juliette Gafni, Theodora Papanikolaou, Francesco DeGiacomo, Jennifer Holcomb, Sylvia Chen, Menalled L.; Kudwa A.; Jon Fitzpatrick, Miller S.; Ramboz S.; Pasi Tuunanen, Kimmo Lehtimaki, Xiangdong William Yang, Seung Kwak, Larry Park, David Howland, Hyunsun Park, and Lisa Ellerby Caspase - 6 activity in a BAC HD mouse modulates steady state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment.

R6 / 2 mice have been genetically altered with an abnormal gene that makes them produce a harmful fragment of the mutant huntingtin protein

It turns out that faulty processing of the huntingtin «recipe» produces a short, harmful fragment of the huntingtin protein.

Gafni J, Papanikolaou T, DeGiacomo F, Holcomb J, Chen S, Menalled L, Kudwa A, Fitzpatrick J, Miller S, Ramboz S, Tuunanen P, Lehtimaki K, William YX, Kwak S, Park L, Howland D, Park H, Ellerby L.Caspase - 6 activity in a BACHD mouse modulates steady - state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment.

This study helps us to understand a new possible way in which harmful fragments of the huntingtin protein are generated.

It acts as a caspase inhibitor, which means it could prevent the dangerous huntingtin protein from being broken into small enough fragments to enter the cell's nucleus and mess up the switching on and off of important genes.

Up until now, it was understood that special «cleaver» proteins slice up the huntingtin protein, fragments of huntingtin.