Critical regions for assembly of vertebrate nonmuscle myosin II

T Nakasawa, M Takahashi, F Matsuzawa, S Aikawa… - Biochemistry, 2005 - ACS Publications
Myosin II molecules assemble and form filaments through their C-terminal rod region, and
the dynamic filament assembly−disassembly process of nonmuscle myosin II molecules is …

Fabry disease: correlation between structural changes in α-galactosidase, and clinical and biochemical phenotypes

F Matsuzawa, S Aikawa, H Doi, T Okumiya… - Human genetics, 2005 - Springer
Fabry disease comprises classic and variant phenotypes. The former needs early enzyme
replacement therapy, and galactose infusion is effective for some variant cases. Attempts of …

Structural and biochemical studies on Pompe disease and a “pseudodeficiency of acid α-glucosidase”

Y Tajima, F Matsuzawa, S Aikawa, T Okumiya… - Journal of human …, 2007 - nature.com
We constructed structural models of the catalytic domain and the surrounding region of human
wild-type acid α-glucosidase and the enzyme with amino acid substitutions by means of …

Phosphorylation-induced conformational switching of CPI-17 produces a potent myosin phosphatase inhibitor

M Eto, T Kitazawa, F Matsuzawa, S Aikawa… - Structure, 2007 - cell.com
Phosphorylation of endogenous inhibitor proteins for type-1 Ser/Thr phosphatase (PP1)
provides a mechanism for reciprocal coordination of kinase and phosphatase activities. A …

Binding parameters and thermodynamics of the interaction of imino sugars with a recombinant human acid α-glucosidase (alglucosidase alfa): Insight into the complex …

M Yoshimizu, Y Tajima, F Matsuzawa, S Aikawa… - Clinica Chimica …, 2008 - Elsevier
BACKGROUND: Recently, enzyme enhancement therapy (EET) for Pompe disease involving
imino sugars, which act as potential inhibitors of acid α-glucosidases in vitro, to improve …

Structural and immunocytochemical studies on α-N-acetylgalactosaminidase deficiency (Schindler/Kanzaki disease)

H Sakuraba, F Matsuzawa, S Aikawa, M Kotani… - Journal of human …, 2004 - nature.com
α-N-Acetylgalactosaminidase (α-NAGA) deficiency (Schindler/Kanzaki disease) is a clinically
and pathologically heterogeneous genetic disease with a wide spectrum including an early …

Bcl-2 antiapoptotic protein mediates verotoxin II–induced cell death: possible association between Bcl-2 and tissue failure by E. coli O157: H7

A Suzuki, H Doi, F Matsuzawa, S Aikawa… - Genes & …, 2000 - genesdev.cshlp.org
Verotoxin II (VTII: or Shiga-like toxin 2) is a key factor forEscherichia coli O157:H7–induced
multiple tissue failure and contains a pentameric sequence (NWGRI) similar to the Bcl-2 …

Novel missense mutations in the human lysosomal sialidase gene in sialidosis patients and prediction of structural alterations of mutant enzymes

K Itoh, Y Naganawa, F Matsuzawa, S Aikawa… - Journal of human …, 2002 - nature.com
Three novel missense mutations in the human lysosomal sialidase gene causing amino
acid substitutions (P80L, W240R, and P316S) in the coding region were identified in two …

Difference in Clinical Phenotype, Mutation Position, and Structural Change of RNF213 Rare Variants Between Pediatric and Adult Japanese Patients with Moyamoya …

…, K Azuma, A Nakamura, A Fukui, F Matsuzawa… - Translational Stroke …, 2024 - Springer
It is unclear how rare RNF213 variants, other than the p.R4810K founder variant, affect the
clinical phenotype or the function of RNF213 in moyamoya disease (MMD). This study …

Pathogenic variants in the Fibronectin Type III Domain of leptin receptor: Molecular dynamics simulation and structural analysis

T Kato, F Matsuzawa, N Shojima, T Yamauchi - Journal of Molecular …, 2024 - Elsevier
Several case reports have identified leptin receptor (LEPR) variants associated with severe
obesity in humans. However, the structure of LEPR has only been partially understood until …