Proteopedia - User contributions [en]

Group:MUZIC:XIN

2013-02-04T15:24:32Z

Georg Mlynek:

=== Xin actin-binding repeat-containing protein 1 ===

== Introduction ==

'''Xin actin-binding repeat-containing protein 1''' (Alternative name: Cardiomyopathy-associated protein 1,CMYA1,XIN,mXinα) is coded by the gene (Synonyms:CMYA1, XIN) and has an actin-binding domain (ABD). It crosslinks actin filaments and participates in anchoring of membrane proteins. Intraexogic splicing leads to a least three different isoforms (XinA, XinB, XinC).

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== Sequence Annotation ==

The largest isoform of Xin actin-binding repeat-containing protein 1, XinA comprises 1843 amino acids and is divided in several domains.At the N-terminus there is proline-rich region including an EVH1 domain-binding consensus motif, which is shown to be functional in binding type I EVH1 domains of the members of the Ena/VASP protein family<ref name="r1">PMID: 16631741</ref>. The following Xin repeats domain consists of 16 copies of the 16 aa-comprising consensus sequence and can bind to F-actin. This domain is followed by the proline-rich region 2,3,4 containing SH3 domain binding motifs. At the C-terminus there is filamin C-specific binding site (FBS)<ref name="r1">PMID: 16631741</ref>.No high resolution structures are available. Cherepanova O. et al discuss conducted EM-studies on Xin-repeats binding to F-actin. <ref name="r2">PMID: 16384582</ref>
Xirp2 encoded by the gene CMYA3 comprisis also Xin-Repeats. [http://www.uniprot.org/uniprot/Q702N8 UNIPROT (XIRP1_HUMAN)] [http://www.uniprot.org/uniprot/A4UGR9 UNIPROT (XIRP2_HUMAN)]

[[Image:Xin.jpg]]

Figure 1: Schematic overview of XinA. Depicted in red are the prolin-reach (PR) domains 1,2,3 and 4. In yellow the Xin-repeat domain is shown and in green the filamin C-specific binding site.

== Function and Interactions ==
Xin localizes at intercalated discs (ICD, structure at the end of the myocytes that transduce force from the myofibrils via the cell membranes to the extracelluar matrix and neighboring cells) in the adult heart and at the myotendinous junction (MTJ) of skeletal muscle tissue.

Xin and Mena/VASP colocalize with filamin c in ICDs.
It has been shown that Xin directly binds the EVH1 domain proteins Mena and VASP<ref name="r1">PMID: 16631741</ref>, the unique insertion containing Ig domain 20<ref name="r1">PMID: 16631741</ref> and directly binds F-actin <ref name="r4">PMID: 15454575</ref>.

== Pathology ==
Knock out of all Xin isoforms in mice generates only a mild cardiac phenotype. Wanq Q. et al. review Xin repeat-containing proteins and how this protein family promotes ICD maturation and stability for normal cardiac function<ref name="r5">PMID: 22652799</ref>.

== References ==
<references />

Group:MUZIC:XIN

2011-07-12T00:28:18Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-12T00:28:00Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-12T00:25:07Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-12T00:22:28Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-12T00:19:43Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-12T00:18:18Z

Georg Mlynek:

'''Xin actin-binding repeat-containing protein 1''' (Alternative name: Cardiomyopathy-associated protein 1) is coded by the gene (Synonyms:CMYA1, XIN) and has an actin-binding domain (ABD). It crosslinks actin filaments and participates in anchoring of membrane proteins. Intraexogic splicing leads to a least three different isoforms.

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== Sequence Annotation ==

[[Image:Xin.jpg]]

== Interactions with other proteins/ Function ==

Xin and Mena/VASP colocalize with filamin c in [http://en.wikipedia.org/wiki/Intercalated_disc intercalated discs ](ICD, structure at the end of the myocytes that transduce force from the myofibrils via the cell membranes to the extracelluar matrix and neighboring cells) in the adult heart <ref>PMID:19773341</ref>.

Xin directly binds the EVH1 domain proteins Mena and VASP <ref name="pmid16631741">.
Xin binds the unique insertion of filamin c containing Ig domain 20.
Xin directly binds F-actin <ref name="pmid15454575">.

<blockquote>
This section complements the article on [http://www.rcsb.org/pdb/static.do?p=education_discussion/molecule_of_the_month/pdb106_1.html Poly(A) Polymerase] in the
[http://www.rcsb.org/pdb/static.do?p=education_discussion/molecule_of_the_month/index.html Molecule of the Month] Series. See also [[Teaching Scenes, Tutorials, and Educators' Pages]].
</blockquote>

==Additional Resources==
For additional information, see: [[Translation]] 
For additional examples of transferases, see: [[Transferase]]

== References ==
<references/><nowiki>
<references />

Group:MUZIC:XIN

2011-07-12T00:17:25Z

Georg Mlynek:

'''Xin actin-binding repeat-containing protein 1''' (Alternative name: Cardiomyopathy-associated protein 1) is coded by the gene (Synonyms:CMYA1, XIN) and has an actin-binding domain (ABD). It crosslinks actin filaments and participates in anchoring of membrane proteins. Intraexogic splicing leads to a least three different isoforms.

{{TOC limit|limit=2}}
== Sequence Annotation ==

[[Image:Xin.jpg]]

== Interactions with other proteins/ Function ==

Xin and Mena/VASP colocalize with filamin c in [intercalated discs][http://en.wikipedia.org/wiki/Intercalated_disc](ICD, structure at the end of the myocytes that transduce force from the myofibrils via the cell membranes to the extracelluar matrix and neighboring cells) in the adult heart <ref>PMID:19773341</ref>.

Xin directly binds the EVH1 domain proteins Mena and VASP <ref name="pmid16631741">.
Xin binds the unique insertion of filamin c containing Ig domain 20.
Xin directly binds F-actin <ref name="pmid15454575">.

<blockquote>
This section complements the article on [http://www.rcsb.org/pdb/static.do?p=education_discussion/molecule_of_the_month/pdb106_1.html Poly(A) Polymerase] in the
[http://www.rcsb.org/pdb/static.do?p=education_discussion/molecule_of_the_month/index.html Molecule of the Month] Series. See also [[Teaching Scenes, Tutorials, and Educators' Pages]].
</blockquote>

==Additional Resources==
For additional information, see: [[Translation]] 
For additional examples of transferases, see: [[Transferase]]

== References ==
<references/><nowiki>
<references />

Group:MUZIC:XIN

2011-07-12T00:08:58Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-12T00:02:40Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-12T00:02:15Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-12T00:00:54Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-11T23:58:51Z

Georg Mlynek: /* Interactions with other proteins/Function Function */

Group:MUZIC:XIN

2011-07-11T23:57:47Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-11T23:56:38Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-11T23:53:00Z

Georg Mlynek:

File:Xin.jpg

2011-07-11T23:51:12Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-10T10:34:18Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-10T09:37:52Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-10T09:31:51Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:45:17Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:27:58Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:27:03Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:26:31Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:25:52Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:19:15Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:16:03Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:15:05Z

Georg Mlynek:

Group:MUZIC:XIN

2011-07-05T08:14:00Z

Georg Mlynek:

'''Xin actin-binding repeat-containing protein 1''' (Alternative name: Cardiomyopathy-associated protein 1) is coded by the gene (Synonyms:CMYA1, XIN) and has an actin-binding domain (ABD). It crosslinks actin filaments and participates in anchoring of membrane proteins.

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== Sequence Annotation ==

[[Image:3hop.png|left|200px|thumb|Crystal structure of Human Filamin A, [[3hop]]]]
{{STRUCTURE_3hop| PDB=3hop | SIZE=300| SCENE=Filamin/Cv/1 |right|CAPTION=Human Filamin A, [[3hop]] }}
[[Filamin]] '''A (FLNA)''' has an actin-binding domain (ABD). It crosslinks actin filaments and participates in anchoring of membrane proteins. '''Filamin B (FLNB)''' is a human cytoplasmic protein which functions similarly to FLNA and guides proper skeletal development. '''Filamin C (FLNC)''' is functionally similar and contains 3 domains: the N-terminal ABD, the C-terminal dimerization domain (DD) and a membrane glycoprotein-binding domain. The images at the left and at the right correspond to one representative filamin structure, ''i.e.'' the crystal structure of human filamin A ([[3hop]]), it forms a <scene name='Filamin/Cv/2'>dimer</scene> <ref>PMID:19773341</ref>. Two phosphate ions are rendered as space filling objects.

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== Filamin A ==

[[3isw]] – hFLNA repeat 21+CFTR peptide – human 
[[2wfn]], [[3hop]], [[3hor]] – hFLNA ABD 
[[3hoc]] – hFLNA ABD (mutant) 

== Function ==
<ref>PMID:19773341</ref>

== Interactions with other proteins ==

Xin directly binds the EVH1 domain proteins Mena and VASP <ref name="pmid16631741">.

== Clinical significance ==

[[1wlh]], [[1qfh]] – FLN rod domain – ''Dictyostelium discoideum'' 

==References==
<references />

==References==
<references />

http://www.uniprot.org/uniprot/Q702N8

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