Results 51 to 60 of about 14,656 (238)

Prenatal muscle development in a mouse model for the secondary dystroglycanopathies [PDF]

, 2016
The defective glycosylation of α-dystroglycan is associated with a group of muscular dystrophies that are collectively referred to as the secondary dystroglycanopathies.
A Leschziner, A Matsakas, A Yoshida, AM Beedle, AR Raphael, B Xia, B-V BD de, C Godfrey, C Longman, C Whitmore, CA Schneider, D Merrick, D Montarras, DE Michele, DJ Blake, DJ Lefeber, E Hohenester, E Stevens, F Muntoni, H Yin, J Reeuwijk van, J Ross, JB Miller, K Buysse, K Kobayashi, KM Wright, KP Campbell, L Wells, M Brockington, M Brockington, M Kanagawa, M Kanagawa, M Narusawa, M Ontell, MD Henry, MJ Duxson, MM Goddeeris, MR Ackroyd, MR Ackroyd, O Armand, S Chan, S Cirak, S Costanzo Di, S Sato, S Schiaffino, S Sugita, SC Brown, T Roscioli, T Willer, TJ Hawke   +49 more
core   +1 more source

Achilles and tail tendons of perlecan exon 3 null heparan sulphate deficient mice display surprising improvement in tendon tensile properties and altered collagen fibril organisation compared to C57BL/6 wild type mice [PDF]

PeerJ, 2018
The aim of this study was to determine the role of the perlecan (Hspg2) heparan sulphate (HS) side chains on cell and matrix homeostasis in tail and Achilles tendons in 3 and 12 week old Hspg2 exon 3 null HS deficient (Hspg2Δ3 − ∕Δ3 −) and C57 BL/6 Wild ...
Cindy C. Shu, Margaret M. Smith, Richard C. Appleyard, Christopher B. Little, James Melrose   +4 more
doaj   +2 more sources

Genetic Suppression of Basement Membrane Defects in Caenorhabditis elegans by Gain of Function in Extracellular Matrix and Cell-Matrix Attachment Genes. [PDF]

, 2018
Basement membranes are extracellular matrices essential for embryonic development in animals. Peroxidasins are extracellular peroxidases implicated in the unique sulfilimine cross-links between type IV basement membrane collagens. Loss of function in the
Chisholm, Andrew D, Gotenstein, Jennifer R, Ho, Tiffany W, Koo, Cassidy C   +3 more
core   +2 more sources

Gabapentin Disrupts Binding of Perlecan to the α₂δ₁ Voltage Sensitive Calcium Channel Subunit and Impairs Skeletal Mechanosensation

Biomolecules, 2022
Our understanding of how osteocytes, the principal mechanosensors within bone, sense and perceive force remains unclear. Previous work identified “tethering elements” (TEs) spanning the pericellular space of osteocytes and transmitting mechanical ...
Perla C. Reyes Fernandez, Christian S. Wright, Adrianna N. Masterson, Xin Yi, Tristen V. Tellman, Andrei Bonteanu, Katie Rust, Megan L. Noonan, Kenneth E. White, Karl J. Lewis, Uma Sankar, Julia M. Hum, Gregory Bix, Danielle Wu, Alexander G. Robling, Rajesh Sardar, Mary C. Farach-Carson, William R. Thompson   +17 more
doaj   +1 more source

Perlecan in late stages of osteoarthritis of the human knee joint [PDF]

Osteoarthritis and Cartilage, 2004
Disturbances of the proteoglycan metabolism play an essential role in the pathology of osteoarthritis. The extracellular matrix proteoglycan, perlecan, has lately been identified as a cell biological factor in cartilage development and maintenance. We investigated the tissue distribution of perlecan, the relation between the level of the protein and ...
Florian Tesche, Nicolai Miosge
openalex   +4 more sources

Heparan Sulfate of Perlecan Is Involved in Glomerular Filtration [PDF]

Journal of the American Society of Nephrology, 2005
Perlecan is a heparan sulfate proteoglycan and a major component of the glomerular basement membrane. To understand the role of heparan sulfate chains of perlecan in glomerular filtration, detailed analyses were performed of the kidneys of
Hideto Watanabe, Raija Soininen, Terukuni Ideura, Hiroyuki Morita, Ling Wang, Kiyoko Inui, Karl Tryggvason, Ashio Yoshimura   +7 more
openaire   +3 more sources

Chemical chaperone treatment reduces intracellular accumulation of mutant collagen IV and ameliorates the cellular phenotype of a COL4A2 mutation that causes haemorrhagic stroke [PDF]

, 2013
Haemorrhagic stroke accounts for approximately 20% of stroke cases and porencephaly is a clinical consequence of perinatal cerebral haemorrhaging. Here we report the identification of a novel dominant G702D mutation in the collagen domain of COL4A2 ...
Aislynn Taggart, Bateman, Brazel, Catheline Vilain, de Clerck, Engin, Favor, Geesin, Gould, Gould, Gould, Hostikka, Ishida, Jeanne, Kalluri, Karl E. Kadler, Khoshnoodi, Lydia S. Murray, Marc Abramowicz, Muthukumaran, Nicole Van Regemorter, Ozcan, Plaisier, Plaisier, Poschl, Ron, Taylor, Tom Van Agtmael, Vahedi, Van Agtmael, Van Agtmael, Van Agtmael, Verbeek, Vilain, Woo, Yinhui Lu   +35 more
core   +1 more source

Perlecan is required for FGF-2 signaling in the neural stem cell niche

Stem Cell Research, 2014
In the adult subventricular zone (neurogenic niche), neural stem cells double-positive for two markers of subsets of neural stem cells in the adult central nervous system, glial fibrillary acidic protein and CD133, lie in proximity to fractones and to ...
Aurelien Kerever, Frederic Mercier, Risa Nonaka, Susana de Vega, Yuka Oda, Bernard Zalc, Yohei Okada, Nobutaka Hattori, Yoshihiko Yamada, Eri Arikawa-Hirasawa   +9 more
doaj   +1 more source

The CS sulphation motifs 4C3, 7D4, 3B3[-]; and perlecan identify stem cell populations and niches, activated progenitor cells and transitional tissue development in the fetal human elbow [PDF]

, 2016
We compared the immunohistochemical distribution of (i) the novel chondroitin sulphate (CS) sulphation motifs 7D4, 4C3 and 3B3[-], (ii) native heparan sulphate (HS) and Δ-HS ‘stubs’ generated by heparitinase III digestion and (iii) the HS-proteoglycan ...
Caterson, Bruce, Hayes, Anthony Joseph, Hughes, Clare Elizabeth, Little, Christopher B., Melrose, James, Smith, Susan M.   +5 more
core   +1 more source

EphA2 is a functional receptor for the growth factor progranulin. [PDF]

, 2016
Although the growth factor progranulin was discovered more than two decades ago, the functional receptor remains elusive. Here, we discovered that EphA2, a member of the large family of Ephrin receptor tyrosine kinases, is a functional signaling receptor
Ahmed, Al-Ayadhi, Andrea Morrione, Atul Goyal, Baker, Bateman, Bhandari, Bhandari, Bogan, Brantley, Brantley-Sieders, Brantley-Sieders, Brantley-Sieders, Brantley-Sieders, Buraschi, Carrasquillo, Catherine Sharpe, Cenik, Chan, Chen, Cheng, Cruts, Cruts, Culouscou, Dave, Dunn, Duxbury, Elizabeth Natkanski, Farach-Carson, Ferluga, Gass, Ghidoni, Gijselinck, Giorgio, Goldoni, Gonzalez, Grindel, He, He, Hess, Hu, Huang, Iozzo, Iozzo, Jun, Kessenbrock, Kohlschütter, Kullander, Lema Tomé, Lemmon, Liliana Schaefer, Lord, Lord, Lovat, Margaryan, Matsubara, Merline, Miao, Minami, Misaki, Monami, Monami, Noberini, Nykjaer, Ogawa, Park, Parri, Petty, Prudencio, Renato V. Iozzo, Romanello, Serrero, Simone Buraschi, Sleegers, Smith, Song, Taddei, Tang, Tangkeangsirisin, Tanimoto, Tanimoto, Thomas Neill, Toh, Toh, Tolkatchev, Van Damme, Van Kampen, Wang, Wesa, Whitelock, Wienken, Wilusz, Wykosky, Wykosky, Xia, Xu, Xu, Yamaguchi, Yang, Zanocco-Marani, Zinchuk, Zoeller   +101 more
core   +2 more sources