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<!-- The PBB_Controls template provides controls for Protein Box Bot, please see Template:PBB_Controls for details. -->
{{Infobox_gene}}
{{PBB_Controls
'''Nebulin-related-anchoring protein''' '''(N-RAP)''' is a [[protein]] that in humans is encoded by the ''NRAP'' [[gene]]. N-RAP is a muscle-specific [[isoform]] belonging to the [[nebulin]] family of [[protein]]s. This family is composed of 5 members: N-RAP, [[nebulin]], [[nebulette]], [[LASP1|LASP-1]] and LASP-2. N-RAP is involved in both myofibrillar myogenesis during development and cell-cell connections in mature muscle.<ref name="pmid12789664">{{cite journal | vauthors = Mohiddin SA, Lu S, Cardoso JP, Carroll S, Jha S, Horowits R, Fananapazir L | title = Genomic organization, alternative splicing, and expression of human and mouse N-RAP, a nebulin-related LIM protein of striated muscle | journal = Cell Motility and the Cytoskeleton | volume = 55 | issue = 3 | pages = 200–12 | date = Jul 2003 | pmid = 12789664 | pmc =  | doi = 10.1002/cm.10123 }}</ref><ref name="pmid10320340">{{cite journal | vauthors = Luo G, Herrera AH, Horowits R | title = Molecular interactions of N-RAP, a nebulin-related protein of striated muscle myotendon junctions and intercalated disks | journal = Biochemistry | volume = 38 | issue = 19 | pages = 6135–43 | date = May 1999 | pmid = 10320340 | pmc =  | doi = 10.1021/bi982395t }}</ref><ref name="entrez">{{cite web | title = Entrez Gene: NRAP nebulin-related anchoring protein| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=4892| accessdate = }}</ref><ref name="pmid20951588">{{cite journal | vauthors = Pappas CT, Bliss KT, Zieseniss A, Gregorio CC | title = The Nebulin family: an actin support group | journal = Trends in Cell Biology | volume = 21 | issue = 1 | date = Jan 2011 | pmid = 20951588 | doi = 10.1016/j.tcb.2010.09.005 | pages=29–37 | pmc=3014390}}</ref>
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<!-- The GNF_Protein_box is automatically maintained by Protein Box Bot. See Template:PBB_Controls to Stop updates. -->
== Structure ==
{{GNF_Protein_box
N-RAP is a 197 kDa protein composed of 1730 amino acids.<ref name="url_COPaKB">{{cite web | url = http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=Q86VF7 | work = Cardiac Organellar Protein Atlas Knowledgebase (COPaKB) | title  = Nebulin-related-anchoring protein }}</ref><ref>{{Cite journal
  | image =
  | pmid = 23965338
| image_source =
| pmc = 4076475
| PDB =  
| year = 2013
| Name = Nebulin-related anchoring protein
| author1 = Zong
| HGNCid = 7988
| first1 = N. C.
| Symbol = NRAP
| title = Integration of cardiac proteome biology and medicine by a specialized knowledgebase
| AltSymbols =;
| journal = Circulation Research
| OMIM = 602873
| volume = 113
| ECnumber =
| issue = 9
| Homologene = 4499
| pages = 1043–53
| MGIid = 1098765
| last2 = Li
| GeneAtlas_image1 = PBB_GE_NRAP_207089_at_tn.png
| first2 = H
| Function = {{GNF_GO|id=GO:0003779 |text = actin binding}} {{GNF_GO|id=GO:0005515 |text = protein binding}} {{GNF_GO|id=GO:0008270 |text = zinc ion binding}} {{GNF_GO|id=GO:0046872 |text = metal ion binding}} {{GNF_GO|id=GO:0051371 |text = muscle alpha-actinin binding}}
| last3 = Li
| Component = {{GNF_GO|id=GO:0005916 |text = fascia adherens}} {{GNF_GO|id=GO:0005927 |text = muscle tendon junction}}
| first3 = H
| Process = {{GNF_GO|id=GO:0008150 |text = biological_process}}
| last4 = Lam
| Orthologs = {{GNF_Ortholog_box
| first4 = M. P.
    | Hs_EntrezGene = 4892
| last5 = Jimenez
    | Hs_Ensembl = ENSG00000197893
| first5 = R. C.
    | Hs_RefseqProtein = NP_006166
| last6 = Kim
    | Hs_RefseqmRNA = NM_006175
| first6 = C. S.
    | Hs_GenLoc_db =
| last7 = Deng
    | Hs_GenLoc_chr = 10
| first7 = N
    | Hs_GenLoc_start = 115338465
| last8 = Kim
    | Hs_GenLoc_end = 115413876
| first8 = A. K.
    | Hs_Uniprot = Q86VF7
| last9 = Choi
    | Mm_EntrezGene = 18175
| first9 = J. H.
    | Mm_Ensembl = ENSMUSG00000049134
| last10 = Zelaya
    | Mm_RefseqmRNA = NM_008733
| first10 = I
    | Mm_RefseqProtein = NP_032759
| last11 = Liem
    | Mm_GenLoc_db =
| first11 = D
    | Mm_GenLoc_chr = 19
| last12 = Meyer
    | Mm_GenLoc_start = 56373351
| first12 = D
    | Mm_GenLoc_end = 56443285
| last13 = Odeberg
    | Mm_Uniprot = Q80XB4
| first13 = J
  }}
| last14 = Fang
}}
| first14 = C
'''Nebulin-related anchoring protein''', also known as '''NRAP''', is a human [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: NRAP nebulin-related anchoring protein| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=4892| accessdate = }}</ref>
| last15 = Lu
| first15 = H. J.
| last16 = Xu
| first16 = T
| last17 = Weiss
| first17 = J
| last18 = Duan
| first18 = H
| last19 = Uhlen
| first19 = M
| last20 = Yates Jr
| first20 = 3rd
| last21 = Apweiler
| first21 = R
| last22 = Ge
| first22 = J
| last23 = Hermjakob
| first23 = H
| last24 = Ping
| first24 = P
| doi = 10.1161/CIRCRESAHA.113.301151
}}</ref> As a member of the nebulin family of proteins, N-RAP is characterized by 35 amino acid stretches of ‘‘nebulin repeats’’, which are actin binding domains containing a conserved [[serine|S]][[aspartate|D]]xx[[tyrosine|Y]][[lysine|K]] motif.<ref>{{cite journal | vauthors = Labeit S, Gibson T, Lakey A, Leonard K, Zeviani M, Knight P, Wardale J, Trinick J | title = Evidence that nebulin is a protein-ruler in muscle thin filaments | journal = FEBS Letters | volume = 282 | issue = 2 | date = May 1991 | pmid = 2037050 | doi = 10.1016/0014-5793(91)80503-u | pages=313–6}}</ref> Like [[nebulin]], groups of seven single repeats within N-RAP form “super repeats”, which incorporate a single conserved motif [[Tryptophan|W]][[Leucine|L]][[Lysine|K]][[Glycine|G]][[Isoleucine|I]][[Glycine|G]][[Tryptophan|W]] at the end of the third repeat.<ref name="pmid7739042">{{cite journal | vauthors = Labeit S, Kolmerer B | title = The complete primary structure of human nebulin and its correlation to muscle structure | journal = Journal of Molecular Biology | volume = 248 | issue = 2 | date = Apr 1995 | pmid = 7739042 | doi = 10.1016/s0022-2836(95)80052-2 | pages=308–15}}</ref> A unique feature of NRAP relative to [[nebulin]] is its N-terminal [[cysteine]]-rich [[LIM domain]], a feature shared with [[LASP1|LASP-1]] and LASP-2.<ref name="pmid20951588"/>


<!-- The PBB_Summary template is automatically maintained by Protein Box Bot. See Template:PBB_Controls to Stop updates. -->
== Function ==
{{PBB_Summary
An important role has been implicated for N-RAP in myofibrilar organization during cardiomyocyte development. It is clear that NRAP is critical for normal α-actinin-dependent organization of myofibrils in cardiomyocytes, as knock-down of N-RAP protein levels causes myofbrillar disassembly in embryonic cardiomyocytes.<ref name="pmid16767749">{{cite journal | vauthors = Dhume A, Lu S, Horowits R | title = Targeted disruption of N-RAP gene function by RNA interference: a role for N-RAP in myofibril organization | journal = Cell Motility and the Cytoskeleton | volume = 63 | issue = 8 | date = Aug 2006 | pmid = 16767749 | doi = 10.1002/cm.20141 | pages=493–511}}</ref> Specifically, studies suggest that NRAP super repeats may be an essential scaffold for organizing [[ACTN2|alpha-actinin]] and [[ACTC1|actin]] into [[sarcomere]]ic [[sarcomere|I-Z-I]] complexes in premyofibrils,<ref name="pmid14657273">{{cite journal | vauthors = Carroll S, Lu S, Herrera AH, Horowits R | title = N-RAP scaffolds I-Z-I assembly during myofibrillogenesis in cultured chick cardiomyocytes | journal = Journal of Cell Science | volume = 117 | issue = Pt 1 | date = Jan 2004 | pmid = 14657273 | doi = 10.1242/jcs.00847 | pages=105–14}}</ref> and dynamic imaging studies have shown that N-RAP departs from the I-Z-I complexes upon completion of actin thin filament assembly.<ref name="pmid19233165">{{cite journal | vauthors = Manisastry SM, Zaal KJ, Horowits R | title = Myofibril assembly visualized by imaging N-RAP, alpha-actinin, and actin in living cardiomyocytes | journal = Experimental Cell Research | volume = 315 | issue = 12 | date = Jul 2009 | pmid = 19233165 | doi = 10.1016/j.yexcr.2009.02.006 | pages=2126–39 | pmc=2742992}}</ref> In adult cardiac muscle, N-RAP colocalizes to [[intercalated discs]],<ref name="pmid15765519">{{cite journal | vauthors = Lu S, Borst DE, Horowits R | title = N-RAP expression during mouse heart development | journal = Developmental Dynamics | volume = 233 | issue = 1 | date = May 2005 | pmid = 15765519 | doi = 10.1002/dvdy.20314 | pages=201–12}}</ref> where it functions to anchor terminal [[ACTC1|actin]] filaments to the [[sarcolemma]]. It has been suggested that its role in adult muscle is force transduction from the sarcomere to the extracellular matrix.<ref name="pmid9295142">{{cite journal | vauthors = Luo G, Zhang JQ, Nguyen TP, Herrera AH, Paterson B, Horowits R | title = Complete cDNA sequence and tissue localization of N-RAP, a novel nebulin-related protein of striated muscle | journal = Cell Motility and the Cytoskeleton | volume = 38 | issue = 1 | pmid = 9295142 | doi = 10.1002/(SICI)1097-0169(1997)38:1<75::AID-CM7>3.0.CO;2-G | year=1997 | pages=75–90}}</ref>
| section_title =  
| summary_text =  
}}


==References==
== Clinical significance ==
{{reflist|2}}
Though no known direct link exists between N-RAP mutations and human cardiomyopathies, N-RAP has been shown to be significantly upregulated in murine models of [[dilated cardiomyopathy]].<ref name="pmid9421465">{{cite journal | vauthors = Sussman MA, Welch S, Cambon N, Klevitsky R, Hewett TE, Price R, Witt SA, Kimball TR | title = Myofibril degeneration caused by tropomodulin overexpression leads to dilated cardiomyopathy in juvenile mice | journal = The Journal of Clinical Investigation | volume = 101 | issue = 1 | date = Jan 1998 | pmid = 9421465 | doi = 10.1172/JCI1167 | pages=51–61 | pmc=508539}}</ref><ref name="pmid11352937">{{cite journal | vauthors = Ehler E, Horowits R, Zuppinger C, Price RL, Perriard E, Leu M, Caroni P, Sussman M, Eppenberger HM, Perriard JC | title = Alterations at the intercalated disk associated with the absence of muscle LIM protein | journal = The Journal of Cell Biology | volume = 153 | issue = 4 | date = May 2001 | pmid = 11352937 | doi = 10.1083/jcb.153.4.763 | pages=763–72 | pmc=2192386}}</ref> This has been hypothesized to be an adaptive response to correct for disorganized actin thin filament architecture at [[intercalated disc]] junctions in cardiomyocytes during [[dilated cardiomyopathy]].
==Further reading==
 
{{refbegin | 2}}
== References ==
{{PBB_Further_reading
{{reflist|33em}}
| citations =
 
*{{cite journal | author=Lanfranchi G, Muraro T, Caldara F, ''et al.'' |title=Identification of 4370 expressed sequence tags from a 3'-end-specific cDNA library of human skeletal muscle by DNA sequencing and filter hybridization. |journal=Genome Res. |volume=6 |issue= 1 |pages= 35-42 |year= 1996 |pmid= 8681137 |doi= }}
== Further reading ==
*{{cite journal | author=Luo G, Zhang JQ, Nguyen TP, ''et al.'' |title=Complete cDNA sequence and tissue localization of N-RAP, a novel nebulin-related protein of striated muscle. |journal=Cell Motil. Cytoskeleton |volume=38 |issue= 1 |pages= 75-90 |year= 1997 |pmid= 9295142 |doi= 10.1002/(SICI)1097-0169(1997)38:1<75::AID-CM7>3.0.CO;2-G }}
{{refbegin|33em}}
*{{cite journal | author=Luo G, Leroy E, Kozak CA, ''et al.'' |title=Mapping of the gene (NRAP) encoding N-RAP in the mouse and human genomes. |journal=Genomics |volume=45 |issue= 1 |pages= 229-32 |year= 1997 |pmid= 9339382 |doi= 10.1006/geno.1997.4917 }}
* {{cite journal | vauthors = Lanfranchi G, Muraro T, Caldara F, Pacchioni B, Pallavicini A, Pandolfo D, Toppo S, Trevisan S, Scarso S, Valle G | title = Identification of 4370 expressed sequence tags from a 3'-end-specific cDNA library of human skeletal muscle by DNA sequencing and filter hybridization | journal = Genome Research | volume = 6 | issue = 1 | pages = 35–42 | date = Jan 1996 | pmid = 8681137 | doi = 10.1101/gr.6.1.35 }}
*{{cite journal | author=Luo G, Herrera AH, Horowits R |title=Molecular interactions of N-RAP, a nebulin-related protein of striated muscle myotendon junctions and intercalated disks. |journal=Biochemistry |volume=38 |issue= 19 |pages= 6135-43 |year= 1999 |pmid= 10320340 |doi= 10.1021/bi982395t }}
* {{cite journal | vauthors = Luo G, Zhang JQ, Nguyen TP, Herrera AH, Paterson B, Horowits R | title = Complete cDNA sequence and tissue localization of N-RAP, a novel nebulin-related protein of striated muscle | journal = Cell Motility and the Cytoskeleton | volume = 38 | issue = 1 | pages = 75–90 | year = 1997 | pmid = 9295142 | doi = 10.1002/(SICI)1097-0169(1997)38:1<75::AID-CM7>3.0.CO;2-G }}
*{{cite journal  | author=Hartley JL, Temple GF, Brasch MA |title=DNA cloning using in vitro site-specific recombination. |journal=Genome Res. |volume=10 |issue= 11 |pages= 1788-95 |year= 2001 |pmid= 11076863 |doi= }}
* {{cite journal | vauthors = Luo G, Leroy E, Kozak CA, Polymeropoulos MH, Horowits R | title = Mapping of the gene (NRAP) encoding N-RAP in the mouse and human genomes | journal = Genomics | volume = 45 | issue = 1 | pages = 229–32 | date = Oct 1997 | pmid = 9339382 | doi = 10.1006/geno.1997.4917 }}
*{{cite journal | author=Wiemann S, Weil B, Wellenreuther R, ''et al.'' |title=Toward a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs. |journal=Genome Res. |volume=11 |issue= 3 |pages= 422-35 |year= 2001 |pmid= 11230166 |doi= 10.1101/gr.154701 }}
* {{cite journal | vauthors = Hartley JL, Temple GF, Brasch MA | title = DNA cloning using in vitro site-specific recombination | journal = Genome Research | volume = 10 | issue = 11 | pages = 1788–95 | date = Nov 2000 | pmid = 11076863 | pmc = 310948 | doi = 10.1101/gr.143000 }}
*{{cite journal | author=Strausberg RL, Feingold EA, Grouse LH, ''et al.'' |title=Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=99 |issue= 26 |pages= 16899-903 |year= 2003 |pmid= 12477932 |doi= 10.1073/pnas.242603899 }}
* {{cite journal | vauthors = Wiemann S, Weil B, Wellenreuther R, Gassenhuber J, Glassl S, Ansorge W, Böcher M, Blöcker H, Bauersachs S, Blum H, Lauber J, Düsterhöft A, Beyer A, Köhrer K, Strack N, Mewes HW, Ottenwälder B, Obermaier B, Tampe J, Heubner D, Wambutt R, Korn B, Klein M, Poustka A | title = Toward a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs | journal = Genome Research | volume = 11 | issue = 3 | pages = 422–35 | date = Mar 2001 | pmid = 11230166 | pmc = 311072 | doi = 10.1101/gr.GR1547R }}
*{{cite journal  | author=Mohiddin SA, Lu S, Cardoso JP, ''et al.'' |title=Genomic organization, alternative splicing, and expression of human and mouse N-RAP, a nebulin-related LIM protein of striated muscle. |journal=Cell Motil. Cytoskeleton |volume=55 |issue= 3 |pages= 200-12 |year= 2004 |pmid= 12789664 |doi= 10.1002/cm.10123 }}
* {{cite journal | vauthors = Wiemann S, Arlt D, Huber W, Wellenreuther R, Schleeger S, Mehrle A, Bechtel S, Sauermann M, Korf U, Pepperkok R, Sültmann H, Poustka A | title = From ORFeome to biology: a functional genomics pipeline | journal = Genome Research | volume = 14 | issue = 10B | pages = 2136–44 | date = Oct 2004 | pmid = 15489336 | pmc = 528930 | doi = 10.1101/gr.2576704 }}
*{{cite journal  | author=Ota T, Suzuki Y, Nishikawa T, ''et al.'' |title=Complete sequencing and characterization of 21,243 full-length human cDNAs. |journal=Nat. Genet. |volume=36 |issue= 1 |pages= 40-5 |year= 2004 |pmid= 14702039 |doi= 10.1038/ng1285 }}
* {{cite journal | vauthors = Witt SH, Granzier H, Witt CC, Labeit S | title = MURF-1 and MURF-2 target a specific subset of myofibrillar proteins redundantly: towards understanding MURF-dependent muscle ubiquitination | journal = Journal of Molecular Biology | volume = 350 | issue = 4 | pages = 713–22 | date = Jul 2005 | pmid = 15967462 | doi = 10.1016/j.jmb.2005.05.021 }}
*{{cite journal  | author=Deloukas P, Earthrowl ME, Grafham DV, ''et al.'' |title=The DNA sequence and comparative analysis of human chromosome 10. |journal=Nature |volume=429 |issue= 6990 |pages= 375-81 |year= 2004 |pmid= 15164054 |doi= 10.1038/nature02462 }}
* {{cite journal | vauthors = Mehrle A, Rosenfelder H, Schupp I, del Val C, Arlt D, Hahne F, Bechtel S, Simpson J, Hofmann O, Hide W, Glatting KH, Huber W, Pepperkok R, Poustka A, Wiemann S | title = The LIFEdb database in 2006 | journal = Nucleic Acids Research | volume = 34 | issue = Database issue | pages = D415–8 | date = Jan 2006 | pmid = 16381901 | pmc = 1347501 | doi = 10.1093/nar/gkj139 }}
*{{cite journal  | author=Gerhard DS, Wagner L, Feingold EA, ''et al.'' |title=The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). |journal=Genome Res. |volume=14 |issue= 10B |pages= 2121-7 |year= 2004 |pmid= 15489334 |doi= 10.1101/gr.2596504 }}
*{{cite journal  | author=Wiemann S, Arlt D, Huber W, ''et al.'' |title=From ORFeome to biology: a functional genomics pipeline. |journal=Genome Res. |volume=14 |issue= 10B |pages= 2136-44 |year= 2004 |pmid= 15489336 |doi= 10.1101/gr.2576704 }}
*{{cite journal | author=Witt SH, Granzier H, Witt CC, Labeit S |title=MURF-1 and MURF-2 target a specific subset of myofibrillar proteins redundantly: towards understanding MURF-dependent muscle ubiquitination. |journal=J. Mol. Biol. |volume=350 |issue= 4 |pages= 713-22 |year= 2005 |pmid= 15967462 |doi= 10.1016/j.jmb.2005.05.021 }}
*{{cite journal | author=Mehrle A, Rosenfelder H, Schupp I, ''et al.'' |title=The LIFEdb database in 2006. |journal=Nucleic Acids Res. |volume=34 |issue= Database issue |pages= D415-8 |year= 2006 |pmid= 16381901 |doi= 10.1093/nar/gkj139 }}
}}
{{refend}}
{{refend}}


{{protein-stub}}
== External links ==
{{WikiDoc Sources}}
* {{cite web | url = http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=Q86VF7 | work = Cardiac Organellar Protein Atlas Knowledgebase (COPaKB)  | title = Mass spectrometry characterization of human NRAP }}.<ref name="pmid23965338">{{cite journal | vauthors = Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, Deng N, Kim AK, Choi JH, Zelaya I, Liem D, Meyer D, Odeberg J, Fang C, Lu HJ, Xu T, Weiss J, Duan H, Uhlen M, Yates JR, Apweiler R, Ge J, Hermjakob H, Ping P | title = Integration of cardiac proteome biology and medicine by a specialized knowledgebase | journal = Circulation Research | volume = 113 | issue = 9 | date = Oct 2013 | pmid = 23965338 | doi = 10.1161/CIRCRESAHA.113.301151 | pmc=4076475 | pages=1043–1053}}</ref>

Latest revision as of 13:14, 5 September 2017

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Identifiers
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External IDsGeneCards: [1]
Orthologs
SpeciesHumanMouse
Entrez

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Ensembl

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Wikidata
View/Edit Human

Nebulin-related-anchoring protein (N-RAP) is a protein that in humans is encoded by the NRAP gene. N-RAP is a muscle-specific isoform belonging to the nebulin family of proteins. This family is composed of 5 members: N-RAP, nebulin, nebulette, LASP-1 and LASP-2. N-RAP is involved in both myofibrillar myogenesis during development and cell-cell connections in mature muscle.[1][2][3][4]

Structure

N-RAP is a 197 kDa protein composed of 1730 amino acids.[5][6] As a member of the nebulin family of proteins, N-RAP is characterized by 35 amino acid stretches of ‘‘nebulin repeats’’, which are actin binding domains containing a conserved SDxxYK motif.[7] Like nebulin, groups of seven single repeats within N-RAP form “super repeats”, which incorporate a single conserved motif WLKGIGW at the end of the third repeat.[8] A unique feature of NRAP relative to nebulin is its N-terminal cysteine-rich LIM domain, a feature shared with LASP-1 and LASP-2.[4]

Function

An important role has been implicated for N-RAP in myofibrilar organization during cardiomyocyte development. It is clear that NRAP is critical for normal α-actinin-dependent organization of myofibrils in cardiomyocytes, as knock-down of N-RAP protein levels causes myofbrillar disassembly in embryonic cardiomyocytes.[9] Specifically, studies suggest that NRAP super repeats may be an essential scaffold for organizing alpha-actinin and actin into sarcomereic I-Z-I complexes in premyofibrils,[10] and dynamic imaging studies have shown that N-RAP departs from the I-Z-I complexes upon completion of actin thin filament assembly.[11] In adult cardiac muscle, N-RAP colocalizes to intercalated discs,[12] where it functions to anchor terminal actin filaments to the sarcolemma. It has been suggested that its role in adult muscle is force transduction from the sarcomere to the extracellular matrix.[13]

Clinical significance

Though no known direct link exists between N-RAP mutations and human cardiomyopathies, N-RAP has been shown to be significantly upregulated in murine models of dilated cardiomyopathy.[14][15] This has been hypothesized to be an adaptive response to correct for disorganized actin thin filament architecture at intercalated disc junctions in cardiomyocytes during dilated cardiomyopathy.

References

  1. ↑ Mohiddin SA, Lu S, Cardoso JP, Carroll S, Jha S, Horowits R, Fananapazir L (Jul 2003). "Genomic organization, alternative splicing, and expression of human and mouse N-RAP, a nebulin-related LIM protein of striated muscle". Cell Motility and the Cytoskeleton. 55 (3): 200–12. doi:10.1002/cm.10123. PMID 12789664.
  2. ↑ Luo G, Herrera AH, Horowits R (May 1999). "Molecular interactions of N-RAP, a nebulin-related protein of striated muscle myotendon junctions and intercalated disks". Biochemistry. 38 (19): 6135–43. doi:10.1021/bi982395t. PMID 10320340.
  3. ↑ "Entrez Gene: NRAP nebulin-related anchoring protein".
  4. ↑ 4.0 4.1 Pappas CT, Bliss KT, Zieseniss A, Gregorio CC (Jan 2011). "The Nebulin family: an actin support group". Trends in Cell Biology. 21 (1): 29–37. doi:10.1016/j.tcb.2010.09.005. PMC 3014390. PMID 20951588.
  5. ↑ "Nebulin-related-anchoring protein". Cardiac Organellar Protein Atlas Knowledgebase (COPaKB).
  6. ↑ Zong, N. C.; Li, H; Li, H; Lam, M. P.; Jimenez, R. C.; Kim, C. S.; Deng, N; Kim, A. K.; Choi, J. H.; Zelaya, I; Liem, D; Meyer, D; Odeberg, J; Fang, C; Lu, H. J.; Xu, T; Weiss, J; Duan, H; Uhlen, M; Yates Jr, 3rd; Apweiler, R; Ge, J; Hermjakob, H; Ping, P (2013). "Integration of cardiac proteome biology and medicine by a specialized knowledgebase". Circulation Research. 113 (9): 1043–53. doi:10.1161/CIRCRESAHA.113.301151. PMC 4076475. PMID 23965338.
  7. ↑ Labeit S, Gibson T, Lakey A, Leonard K, Zeviani M, Knight P, Wardale J, Trinick J (May 1991). "Evidence that nebulin is a protein-ruler in muscle thin filaments". FEBS Letters. 282 (2): 313–6. doi:10.1016/0014-5793(91)80503-u. PMID 2037050.
  8. ↑ Labeit S, Kolmerer B (Apr 1995). "The complete primary structure of human nebulin and its correlation to muscle structure". Journal of Molecular Biology. 248 (2): 308–15. doi:10.1016/s0022-2836(95)80052-2. PMID 7739042.
  9. ↑ Dhume A, Lu S, Horowits R (Aug 2006). "Targeted disruption of N-RAP gene function by RNA interference: a role for N-RAP in myofibril organization". Cell Motility and the Cytoskeleton. 63 (8): 493–511. doi:10.1002/cm.20141. PMID 16767749.
  10. ↑ Carroll S, Lu S, Herrera AH, Horowits R (Jan 2004). "N-RAP scaffolds I-Z-I assembly during myofibrillogenesis in cultured chick cardiomyocytes". Journal of Cell Science. 117 (Pt 1): 105–14. doi:10.1242/jcs.00847. PMID 14657273.
  11. ↑ Manisastry SM, Zaal KJ, Horowits R (Jul 2009). "Myofibril assembly visualized by imaging N-RAP, alpha-actinin, and actin in living cardiomyocytes". Experimental Cell Research. 315 (12): 2126–39. doi:10.1016/j.yexcr.2009.02.006. PMC 2742992. PMID 19233165.
  12. ↑ Lu S, Borst DE, Horowits R (May 2005). "N-RAP expression during mouse heart development". Developmental Dynamics. 233 (1): 201–12. doi:10.1002/dvdy.20314. PMID 15765519.
  13. ↑ Luo G, Zhang JQ, Nguyen TP, Herrera AH, Paterson B, Horowits R (1997). "Complete cDNA sequence and tissue localization of N-RAP, a novel nebulin-related protein of striated muscle". Cell Motility and the Cytoskeleton. 38 (1): 75–90. doi:10.1002/(SICI)1097-0169(1997)38:1<75::AID-CM7>3.0.CO;2-G. PMID 9295142.
  14. ↑ Sussman MA, Welch S, Cambon N, Klevitsky R, Hewett TE, Price R, Witt SA, Kimball TR (Jan 1998). "Myofibril degeneration caused by tropomodulin overexpression leads to dilated cardiomyopathy in juvenile mice". The Journal of Clinical Investigation. 101 (1): 51–61. doi:10.1172/JCI1167. PMC 508539. PMID 9421465.
  15. ↑ Ehler E, Horowits R, Zuppinger C, Price RL, Perriard E, Leu M, Caroni P, Sussman M, Eppenberger HM, Perriard JC (May 2001). "Alterations at the intercalated disk associated with the absence of muscle LIM protein". The Journal of Cell Biology. 153 (4): 763–72. doi:10.1083/jcb.153.4.763. PMC 2192386. PMID 11352937.

Further reading

External links

  1. ↑ Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, Deng N, Kim AK, Choi JH, Zelaya I, Liem D, Meyer D, Odeberg J, Fang C, Lu HJ, Xu T, Weiss J, Duan H, Uhlen M, Yates JR, Apweiler R, Ge J, Hermjakob H, Ping P (Oct 2013). "Integration of cardiac proteome biology and medicine by a specialized knowledgebase". Circulation Research. 113 (9): 1043–1053. doi:10.1161/CIRCRESAHA.113.301151. PMC 4076475. PMID 23965338.
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