Blar i Bergen Open Research Archive på forfatter "Ruskamo, Salla"
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Conformational analysis of membrane-proximal segments of GDAP1 in a lipidic environment using synchrotron radiation suggests a mode of assembly at the mitochondrial outer membrane
Sutinen, Aleksi; Jones, Nykola C.; Hoffmann, Søren Vrønning; Ruskamo, Salla; Kursula, Petri Tapani (Journal article; Peer reviewed, 2023)The mitochondrial outer membrane creates a diffusion barrier between the cytosol and the mitochondrial intermembrane space, allowing the exchange of metabolic products, important for efficient mitochondrial function in ... -
Conserved intramolecular networks in GDAP1 are closely connected to CMT-linked mutations and protein stability
Sutinen, Aleksi; Paffenholz, Dirk; Nguyen, Giang Thi Tuyet; Ruskamo, Salla; Torda, Andrew E.; Kursula, Petri Tapani (Journal article; Peer reviewed, 2023)Charcot-Marie-Tooth disease (CMT) is the most common inherited peripheral polyneuropathy in humans, and its subtypes are linked to mutations in dozens of different genes, including the gene coding for ganglioside-induced ... -
Cryo-EM, X-ray diffraction, and atomistic simulations reveal determinants for the formation of a supramolecular myelin-like proteolipid lattice
Ruskamo, Salla; Krokengen, Oda Caspara; Kowal, Julia; Nieminen, Tuomo; Lehtimäki, Mari; Raasakka, Arne; Dandey, Venkata P; Vattulainen, Ilpo; Stahlberg, Henning; Kursula, Petri (Journal article; Peer reviewed, 2020)Myelin protein P2 is a peripheral membrane protein of the fatty acid–binding protein family that functions in the formation and maintenance of the peripheral nerve myelin sheath. Several P2 gene mutations cause human ... -
Crystal and solution structure of NDRG1, a membranebinding protein linked to myelination and tumour suppression
Mustonen, Venla; Muruganandam, Gopinath; Loris, Remy; Kursula, Petri; Ruskamo, Salla (Journal article; Peer reviewed, 2021)N-myc downstream-regulated gene 1 (NDRG1) is a tumour suppressor involved in vesicular trafficking and stress response. NDRG1 participates in peripheral nerve myelination, and mutations in the NDRG1 gene lead to ... -
Human myelin protein P2: from crystallography to time-lapse membrane imaging and neuropathy-associated variants
Uusitalo, Maiju; Klenow, Martin Berg; Laulumaa, Saara; Blakeley, Matthew P.; Simonsen, Adam Cohen; Ruskamo, Salla; Kursula, Petri (Journal article; Peer reviewed, 2021)Peripheral myelin protein 2 (P2) is a fatty acid-binding protein expressed in vertebrate peripheral nervous system myelin, as well as in human astrocytes. Suggested functions of P2 include membrane stacking and lipid ... -
Human myelin proteolipid protein structure and lipid bilayer stacking
Ruskamo, Salla; Raasakka, Arne; Pedersen, Jan Skov; Martel, Anne; Škubník, Karel; Darwish, Tamim; Porcar, Lionel; Kursula, Petri (Journal article; Peer reviewed, 2022)The myelin sheath is an essential, multilayered membrane structure that insulates axons, enabling the rapid transmission of nerve impulses. The tetraspan myelin proteolipid protein (PLP) is the most abundant protein of ... -
Membrane Association Landscape of Myelin Basic Protein Portrays Formation of the Myelin Major Dense Line
Raasakka, Arne; Ruskamo, Salla; Kowal, Julia; Barker, Robert; Baumann, Anne; Martel, Anne; Tuusa, Jussi; Myllykoski, Matti; Bürck, Jochen; Ulrich, Anne S.; Stahlberg, Henning; Kursula, Petri (Peer reviewed; Journal article, 2017-07-10)Compact myelin comprises most of the dry weight of myelin, and its insulative nature is the basis for saltatory conduction of nerve impulses. The major dense line (MDL) is a 3-nm compartment between two cytoplasmic leaflets ... -
Molecular mechanisms of Charcot-Marie-Tooth neuropathy linked to mutations in human myelin protein P2
Ruskamo, Salla; Nieminen, Tuomo; Kristiansen, Cecilie Katrin; Vatne, Guro Helén; Baumann, Anne; Hallin, Erik Ingmar; Raasakka, Arne; Joensuu, Päivi; Bergmann, Ulrich; Vattulainen, Ilpo; Kursula, Petri (Peer reviewed; Journal article, 2017-07-26)Charcot-Marie-Tooth (CMT) disease is one of the most common inherited neuropathies. Recently, three CMT1-associated point mutations (I43N, T51P, and I52T) were discovered in the abundant peripheral myelin protein P2. These ... -
Molecular structure and function of myelin protein P0 in membrane stacking
Raasakka, Arne; Ruskamo, Salla; Kowal, Julia; Han, Huijong; Baumann, Anne; Myllykoski, Matti; Fasano, Anna; Rossano, Rocco; Riccio, Paolo; Bürck, Jochen; Ulrich, Anne S.; Stahlberg, Henning; Kursula, Petri (Peer reviewed; Journal article, 2019)Compact myelin forms the basis of nerve insulation essential for higher vertebrates. Dozens of myelin membrane bilayers undergo tight stacking, and in the peripheral nervous system, this is partially enabled by myelin ... -
Myelin-derived and putative molecular mimic peptides share structural properties in aqueous and membrane-like environments
Tuusa, Jussi; Raasakka, Arne; Ruskamo, Salla; Kursula, Petri (Peer reviewed; Journal article, 2017-03-24)Background: Despite intense research, the causes of various neurological diseases remain enigmatic to date. A role for viral or bacterial infection and associated molecular mimicry has frequently been suggested in the ... -
Neuropathy-related mutations alter the membrane binding properties of the human myelin protein P0 cytoplasmic tail
Raasakka, Arne; Ruskamo, Salla; Barker, Robert; Krokengen, Oda Caspara; Vatne, Guro Helén; Kristiansen, Cecilie Katrin; Hallin, Erik Ingmar; Skoda, Maximilian W. A.; Bergmann, Ulrich; Wacklin-Knecht, Hanna; Jones, Nykola C; Hoffmann, Søren V; Kursula, Petri (Peer reviewed; Journal article, 2019-06-07)Schwann cells myelinate selected axons in the peripheral nervous system (PNS) and contribute to fast saltatory conduction via the formation of compact myelin, in which water is excluded from between tightly adhered lipid ... -
Structural insights into Charcot–Marie–Tooth disease-linked mutations in human GDAP1
Sutinen, Aleksi; Nguyen, Giang Thi Tuyet; Raasakka, Arne; Muruganandam, Gopinath; Loris, Remy; Ylikallio, Emil; Tyynismaa, Henna; Bartesaghi, Luca; Ruskamo, Salla; Kursula, Petri (Journal article; Peer reviewed, 2022)Charcot–Marie–Tooth disease (CMT) is the most common inherited peripheral polyneuropathy in humans, and its different subtypes are linked to mutations in dozens of different genes. Mutations in ganglioside-induced ... -
Structure and dynamics of a human myelin protein P2 portal region mutant indicate opening of the β barrel in fatty acid binding proteins
Laulumaa, Saara; Nieminen, Tuomo; Raasakka, Arne; Krokengen, Oda C.; Safaryan, Anushik; Hallin, Erik Ingmar; Brysbaert, Guillaume; Lensink, Marc F.; Ruskamo, Salla; Vattulainen, Ilpo; Kursula, Petri (Peer reviewed; Journal article, 2018-06-25)Background: Myelin is a multilayered proteolipid sheath wrapped around selected axons in the nervous system. Its constituent proteins play major roles in forming of the highly regular membrane structure. P2 is a myelin-specific ...