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Page 1
Genetic Dominant Variants in STUB1, Segregating in Families with SCA48, Display In Vitro Functional Impairments Indistinctive from Recessive Variants Associated with SCAR16.
Pakdaman Y, Berland S, Bustad HJ, Erdal S, Thompson BA, James PA, Power KN, Ellingsen S, Krooni M, Berge LI, Sexton A, Bindoff LA, Knappskog PM, Johansson S, Aukrust I. Pakdaman Y, et al. Among authors: bustad hj. Int J Mol Sci. 2021 May 30;22(11):5870. doi: 10.3390/ijms22115870. Int J Mol Sci. 2021. PMID: 34070858 Free PMC article.
Arc self-association and formation of virus-like capsids are mediated by an N-terminal helical coil motif.
Eriksen MS, Nikolaienko O, Hallin EI, Grødem S, Bustad HJ, Flydal MI, Merski I, Hosokawa T, Lascu D, Akerkar S, Cuéllar J, Chambers JJ, O'Connell R, Muruganandam G, Loris R, Touma C, Kanhema T, Hayashi Y, Stratton MM, Valpuesta JM, Kursula P, Martinez A, Bramham CR. Eriksen MS, et al. Among authors: bustad hj. FEBS J. 2021 May;288(9):2930-2955. doi: 10.1111/febs.15618. Epub 2020 Nov 28. FEBS J. 2021. PMID: 33175445 Free article.
A Pharmacological Chaperone Therapy for Acute Intermittent Porphyria.
Bustad HJ, Toska K, Schmitt C, Vorland M, Skjærven L, Kallio JP, Simonin S, Letteron P, Underhaug J, Sandberg S, Martinez A. Bustad HJ, et al. Mol Ther. 2020 Feb 5;28(2):677-689. doi: 10.1016/j.ymthe.2019.11.010. Epub 2019 Dec 4. Mol Ther. 2020. PMID: 31810863 Free PMC article.
In vitro characterization of six STUB1 variants in spinocerebellar ataxia 16 reveals altered structural properties for the encoded CHIP proteins.
Pakdaman Y, Sanchez-Guixé M, Kleppe R, Erdal S, Bustad HJ, Bjørkhaug L, Haugarvoll K, Tzoulis C, Heimdal K, Knappskog PM, Johansson S, Aukrust I. Pakdaman Y, et al. Among authors: bustad hj. Biosci Rep. 2017 Apr 28;37(2):BSR20170251. doi: 10.1042/BSR20170251. Print 2017 Apr 30. Biosci Rep. 2017. PMID: 28396517 Free PMC article.
Arc is a flexible modular protein capable of reversible self-oligomerization.
Myrum C, Baumann A, Bustad HJ, Flydal MI, Mariaule V, Alvira S, Cuéllar J, Haavik J, Soulé J, Valpuesta JM, Márquez JA, Martinez A, Bramham CR. Myrum C, et al. Among authors: bustad hj. Biochem J. 2015 May 15;468(1):145-58. doi: 10.1042/BJ20141446. Biochem J. 2015. PMID: 25748042 Free PMC article.
14 results