Artificial β-propeller protein-based hydrolases.
Sign Up to like & getrecommendations! Published in 2019 at "Chemical communications"
DOI: 10.1039/c9cc04388h
Abstract: We developed an artificial hydrolase based on the symmetrical Pizza6 β-propeller protein for the metal-free hydrolysis of 4-nitrophenyl acetate and butyrate. Through site-specific mutagenesis and crystallisation studies, the catalytic mechanism was investigated and found to… read more here.
Keywords: artificial propeller; protein based; propeller; based hydrolases ... See more keywords
Childhood Dystonia-Parkinsonism Following Infantile Spasms-Clinical Clue to Diagnosis in Early Beta-Propeller Protein-Associated Neurodegeneration.
Sign Up to like & getrecommendations! Published in 2019 at "Neuropediatrics"
DOI: 10.1055/s-0039-1696688
Abstract: INTRODUCTION Beta-propeller protein-associated neurodegeneration (BPAN) is a very rare, X-linked dominant (XLD) inherited member of the neurodegeneration with brain iron accumulation (NBIA) disease family. CASE REPORT We present a female case of BPAN with infantile… read more here.
Keywords: symptomatology; infantile spasms; dystonia; neurodegeneration ... See more keywords
A conserved ATG2 binding site in WIPI4 and yeast Hsv2 is disrupted by mutations causing β-propeller protein-associated neurodegeneration.
Sign Up to like & getrecommendations! Published in 2021 at "Human molecular genetics"
DOI: 10.1093/hmg/ddab225
Abstract: PROPPINs are phosphoinositide-binding β-propeller proteins that mediate membrane recruitment of other proteins and are involved in different membrane remodeling processes. The main role of PROPPINs is their function in autophagy, where they act at different… read more here.
Keywords: hsv2; atg2 binding; wipi4; propeller protein ... See more keywords
Expanding the Spectrum of Early Neuroradiologic Findings in β Propeller Protein-Associated Neurodegeneration
Sign Up to like & getrecommendations! Published in 2022 at "American Journal of Neuroradiology"
DOI: 10.3174/ajnr.a7693
Abstract: BACKGROUND AND PURPOSE: β propeller protein-associated neurodegeneration (BPAN) is the most common neurodegeneration with brain iron accumulation disorder. Typical radiologic findings are T2 hypointensity in the substantia nigra and globus pallidus, as well as a… read more here.
Keywords: substantia nigra; protein associated; propeller protein; neurodegeneration ... See more keywords
Ferrous Iron Up-regulation in Fibroblasts of Patients with Beta Propeller Protein-Associated Neurodegeneration (BPAN)
Sign Up to like & getrecommendations! Published in 2017 at "Frontiers in Genetics"
DOI: 10.3389/fgene.2017.00018
Abstract: Mutations in WDR45 gene, coding for a beta-propeller protein, have been found in patients affected by Neurodegeneration with Brain Iron Accumulation, NBIA5 (also known as BPAN). BPAN is a movement disorder with Non Transferrin Bound… read more here.
Keywords: propeller protein; bpan; beta propeller; iron ... See more keywords