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From CellBiology

Syncoilin protein SYNC1 (64 kDa)


  • Syncoilin (SYNC1), an intermediate filament-like associated complex protein in skeletal muscle.
  • Syncoilin protein is an essential ingredient of the complex system of structural protein elements that shape the cytoskeleton cytoskeleton. structural design of muscular tissue and make available the mechanical power and structural association required to transport about movement during contraction and actions of muscle fibres.
  • Syncoilin is significantly associated with the intermediate filament protein category,the protein is greatly present in skeletal and cardiac muscle within the human body. Protein Syncollin Localizes
  • Syncoilin protein attaches to a-dystrobrevin, which is a element of the dystrophin linked protein segment (DAPC) situated adjacent to the cell membrane in muscle cellular material, in addition to desmin, a muscle-related intermediate filament protein SYNC1, consequently linking the DAPC and the muscle cellular intermediate filament complex.
  • This main association possibly will be vital for muscular integrity and strength of specific muscle groups throughout contraction mechanism. Furthermore, Syncoilin is established at amplified stages in the redeveloped muscle segments of individuals with muscular abnormalties and muscle disease. Hence,Syncoilin may perhaps be essential for muscular restoration in response to damage to certain muscular fibres.
  • Syncoilin is a 64-kDa intermediate filament protein is established in skeletal muscular tissue and is developed at the sites of the perinucleus, sarcolemma, and myotendinous and neuromuscular sites (junctions).
  • Structural pattern of the localization and attachment spots of the Syncoilin 64-kDa intermediate filament protein is an ultimate applicant to be in cooperation with a significant structural element of myocytes and a possible mediator of inherited myopathies elements.[4]
Image details: modification by J.Nassif (2009) Primary source:[http://www.jbc.org/cgi/reprint/277/5/3433

Joe Nassif 13:40, 12 May 2009 (EST)


Introduction

  • Syncoilin (SYNC1) protein is a constituent of the intermediate filament group (IF) protein family, decidedly visible among the striated muscle tissue.Protein Syncollin Localizes
  • The specified protein offers a linkage connecting the dystrophin related protein complex as well as the desmin Intermediate Filament association and is re-organised in a assortment of individual myopathies and muscle related diseases.
  • Despite the fact that the configuration, localization and chief attachment sites of syncoilin have been well analyzed, nevertheless yet the function sciencfically remains indistinguishable. SYNCOILIN PROTEIN DETAILS/CONFIGURATION



Structure of Syncoilin

  • Syncoilin (SYNC1) protein is a 64 kDa element, acknowledged as a component of the intermediate filament complex on the foundation of the protein sequence being a homology structure. Conversely, it does not produce filaments segments through homopolymerisation process.
  • Similarly all intermediate filaments,including Syncoilin have a fundamental rod domain designed up of four spiral-coil resulting in the appearance of, a-helical section alienated by flexible connectors, in addition an N-terminal base area and a C-terminal end province, which are structurally lesser distinct.
  • The base domain of Syncoilin tolerates no important homology to any supplementary protein segment.
  • The end (tail) area is oddly undersized, which possibly will clarify part of the Syncoilin which lacks the filament-creating capability, given that end domains of other Intermediate Filaments are essential for accurate fundamental assembly of filaments. [5]
  • The consensus structure of the Intermediate Filament segment are distinct with a heterogenous configuration and pattern in muscle tissue.
Image source: Dr M.Hill[1]
FIGURE A Syncoilin protein is a 64 kDa,Image details: modification by J.Nassif (2009)

Expression and pattern of syncoilin

  • Syncoilin protein is exceedingly situated in skeletal and cardiac cellular muscle cells. Within skeletal muscle tissue, Syncoilin is located all the way through the sarcolemma, although is developed at the neuromuscular junction and myotendinous junctions in muscular tissue segments and in the region surrounding the nucleus.
  • Neighboring the interior aspects of the muscle fibers, Syncoilin is generalized to accommodate the Z-lines sections, through the technique of immunostaining it demonstrate to facilitate a quantity of syncoilin protein additionally are present in soluble material in the cytoplasm of muscular cells. Amusingly, experiments tested on specific species revealed that the Syncoilin protein is redistributed from each and every one of its typical cellular site and completely reallocated to the soluble portion of the cellular material surrounding the cytoplasm, representing that it is reliant on specfic desmin protein substance for its standard positioning and involvement with the cytoskeleton organization.[6]
  • In distinction, Syncoilin localization is unaffected in the muscles cells of a-dystrobrevin in muscles cells, indicating that the relationship to the DAPC is not essential for Syncoilin protein strength at the sarcolemma.
  • Syncoilin related protein is augmented at the sarcolemma of a-dystrobrevin site in human species, almost certainly as a effect of their placid muscular functioning. Syncoilin is distinct further extremely in slow, oxidative fibers than in rapid, fibres and consequently may perhaps contribute to the separate characteristics of slow muscle in contrast to fast fibers.
  • Syncoilin might give rise to the differential contractile apparatus properties of slow fibers within muscular cells, hence decreased limitation velocity and augmented resistance to fatigue situations.

--Joe Nassif 18:20, 20 May 2009 (EST)

Image details: modification by J.Nassif (2009) from primary source:[2]
Syncoilin protein is a 64 kDa, Poon, E.,Howman, E.V.,Newey, S.E. and Davies,K.E. (2002)Association of syncoilin and desmin: linking intermediate filament proteins to the dystrophin-associated protein complex.J. Biol. Chem. 277, 3433–3439.
Image Primary source gathered from: Association of Syncoilin


Syncoilin in relation to muscle disease

  • Syncoilin protein is establish at amplified levels in the muscular tissues of individuals with neuromuscular deformities, together with DMD, congenital muscular dystrophy (CMD) along with desmin-related myopathy.
  • Limited information is acknowledged in respect to Syncoilin in CMD, however the increase of Syncoilin in DMD and desmin-related myopathy has been examined in significantly. Interestingly, desminrelated myopathy is not typically a result due to alteration in cellular material in desmin, although might be established by mutations in a different source of binding sites, for example aB-crystallin . [7]
  • Syncoilin protein sequence alteration can moreover be accountable for a quantity of disease .To date, 76 individuals among desmin-specfic myopathy have been tested for mutations in relations to the Syncoilin protein lacking any such modifications being recognized, however this does not eliminate the optional rare cases .
Image Source:[3]Reference:Werner, D. (1987). Muscular Dystrophy. Retrieved March 26, 2008, from Disabled Village Children Web site


--Joe Nassif 18:20, 20 May 2009 (EST)



Role of syncoilin protein in skeletal muscle

  • Syncoilin protein is an essential ingredient of the complex system of structural proteins elements that shape the cytoskeleton structural design of skeletal muscular tissue and make available the mechanical power and structural association required to transport about movement during contraction actions.Analysis of the association of syncollin protein.
  • The significance of the collection of Syncoilin proteins that connect the DAPC to desmin inrelation to Syncoilin protein established at additional subcellular locations for instance the Z-lines and MTJ, for strength and energy production, involve additional analysis.
  • The failure of Syncoilin elements to produce filaments regardless of its intermediate filament protein domain arrangement and connection with the desmin protein recommends that its purpose might vary from that of other filament component.
  • Accordingly, a potential representation for the relationship of Syncoilin proteins with intermediate filaments is that it combines with the region of filaments, and operates as a molecular adaptor to permit the involvement of additional proteins with the Filament complex.
  • The association to the DAPC can be considered to be an individual case, however Syncoilin may as well provide to employ proteins that persuade the dynamics of the imtermediate system, to connect the complex cytoskeletal elements, to operate as a scaffold to accumulate signal proteins at appropriate subcellular locations or, as discussed above, to play a part in the conveyance of indications itself.Syncollin Skeletal muscle function
  • This design of Syncoilin protein as a adaptor can be associated to the augmented Syncoilin stages presented in the muscular dystrophies and other muscular related diseases. Amplified levels of the Syncoilin Protein adaptor in muscular tissue diseases may excessively be a compensatory reaction, to reinforcing the cytoskeleton structure or to adjust specific indications in an effort to defeat the degeneration of the certain muscles groups.Analysis of the association of syncollin protein.

--Joe Nassif 18:20, 20 May 2009 (EST)



Overview and future directions

  • Syncoilin protein substance is a exceptional, non-filament-forming caterory of the intermediate filament network of protein that assists in connecting the DAPC to desmin filaments in muscular cells and has a keys role in contractile strength communication between muscles.
  • The verification gathered so far put forward that Syncoilin protein may perhaps operate as a molecular adaptor for Intermediate Filaments complexes, and with the intention of it may participate in the main role in muscle renewal.
  • The ongoing examination and testing of the role of the Syncoilin protein may grant essential approaches into the functions of intermediate filament networks in typical muscular disease.Syncollin Skeletal muscle function

--Joe Nassif 18:20, 20 May 2009 (EST)



Syncoilin protein- Related Terminology:


Apoptosis: is a form of programmed cell death in multicellular organisms.

C-terminal: The region of a protein near the end with a free carboxyl group.

Contractile: contractility - the capability or quality of shrinking or contracting, especially by muscle fibers and even some other forms of living matter.

Cytoskeleton: Microscopic network of actin filaments and microtubules in the cytoplasm of many living cells that gives the cell shape and coherence.

Dystrophies: Dystrophy - A wasting of body tissues, of genetic origin or due to inadequate or defective nutrition.

Homology: The quality of being similar or corresponding in position or value or structure or function.

Homopolymerisation: The polymerization of a single type of monomer to form a homopolymer.

Immunostaining:Immunostaining is a general term in biochemistry that applies to any use of an antibody-based method to detect a specific protein in a sample.

Intermediate filament: Intermediate filaments (IFs) are cytoskeletal structures formed by members of a related proteins called keratin.

Localization: The process of determining or marking the location or site of a lesion or disease.

N-terminal: The end of a protein (either the RNA polymerases involved in the process of transcription or the polypeptide product of the DNA).

Perinucleus: Perinuclear - Surrounding a nucleus.

Sarcolemma: an extensible membrane enclosing the contractile substance of a muscle fiber.

Syncoilin protein: Syncoilin is a member of the intermediate filament protein family, highly expressed in skeletal and cardiac muscle.

Reference/Source: WORDNET Definitions "A lexical database for the English language"


Acronyms


CMD- Congenital muscular dystrophy

DAPC- Dystrophin-associated protein complex

IF- Intermediate Filament

EBS- epidermolysis bullosa simplex filensin

GFAP- glial gibrillary acidic protein

IFAP- Intermediate Filament Associated Protein

IFP- Intermediate Filament Protein

KF- Keratin filament

NF- neurofilament

NF-L- neurofilament-low molecular mass protein




References


Textbooks

Molecular Biology of the Cell Alberts, Bruce; Johnson, Alexander; Lewis, Julian; Raff, Martin; Roberts, Keith; Walter, Peter New York and London: Garland Science; c2002

  • Muscle Cells Can Vary Their Properties by Changing the Protein Isoforms They Contain
  • New Skeletal Muscle Fibers Form by the Fusion of Myoblasts
  • Skeletal Muscle Fibers Secrete Myostatin to Limit Their own Growth
  • Skeletal muscle cells (also called muscle fibers)


The Cell- A Molecular Approach Cooper, Geoffrey M. Sunderland (MA): Sinauer Associates, Inc.; c2000

  • Muscle Contraction


PUBMED

  • Analysis of skeletal muscle function in the C57BL6/SV129 syncoilin knockout mouse Syncollin Skeletal muscle function
  • Intermediate filament cytoskeleton of the liver in health and disease[8]
  • INTERMEDIATE FILAMENTS IN SMOOTH MUSCLE[9]

Articles

Analysis of the association of syncollin with the membrane of the pancreatic zymogen granule.An SJ, Hansen NJ, Hodel A, Jahn R, Edwardson JM (2000) . J Biol Chem 275:11306–11311Analysis of the association of syncollin protein.

Syncollin is differentially expressed Am. J. Physiol. Gastrointest. Liver Physiol. 278: G308–G320, 2000. Syncollin is differentially expressed

The Secretory Granule Protein Syncollin Localizes to HL-60 Cells and Neutrophils .An SJ, Hansen NJ, Hodel A, Jahn R, Edwardson JM (2000) Analysis of the association of syncollin with the membrane of the pancreatic zymogen granule. J Biol Chem 275:11306–11311 Protein Syncollin Localizes

Poon EP, Howman EV, Newey SE, Davies KE. Association of syncoilin and desmin: linking intermediate filament proteins to the dystrophin-associated protein complex. J Biol Chem 2002;277:3433–3439.[10]

OMIM entry: SYNCOILIN 1; SYNC1[11]

OMIM entry: DYSTROBREVIN, ALPHA; DTNA [12]

OMIM entry: ACTIN, ALPHA-2, SMOOTH MUSCLE, AORTA; ACTA2 [13]



INDIVIDUAL LECTURE QUESTION WILL BE FOUND ON DISCUSSION PAGE (J.NASSIF,2009)

--Joe Nassif 11:32, 28 May 2009 (EST)