| Tbio | rRNA 2'-O-methyltransferase fibrillarin |
S-adenosyl-L-methionine-dependent methyltransferase that has the ability to methylate both RNAs and proteins. Involved in pre-rRNA processing by catalyzing the site-specific 2'-hydroxyl methylation of ribose moieties in pre-ribosomal RNA. Site specificity is provided by a guide RNA that base pairs with the substrate. Methylation occurs at a characteristic distance from the sequence involved in base pairing with the guide RNA. Also acts as a protein methyltransferase by mediating methylation of 'Gln-105' of histone H2A (H2AQ104me), a modification that impairs binding of the FACT complex and is specifically present at 35S ribosomal DNA locus (PubMed:24352239).
This gene product is a component of a nucleolar small nuclear ribonucleoprotein (snRNP) particle thought to participate in the first step in processing preribosomal RNA. It is associated with the U3, U8, and U13 small nuclear RNAs and is located in the dense fibrillar component (DFC) of the nucleolus. The encoded protein contains an N-terminal repetitive domain that is rich in glycine and arginine residues, like fibrillarins in other species. Its central region resembles an RNA-binding domain and contains an RNP consensus sequence. Antisera from approximately 8% of humans with the autoimmune disease scleroderma recognize fibrillarin. [provided by RefSeq, Jul 2008]
This gene product is a component of a nucleolar small nuclear ribonucleoprotein (snRNP) particle thought to participate in the first step in processing preribosomal RNA. It is associated with the U3, U8, and U13 small nuclear RNAs and is located in the dense fibrillar component (DFC) of the nucleolus. The encoded protein contains an N-terminal repetitive domain that is rich in glycine and arginine residues, like fibrillarins in other species. Its central region resembles an RNA-binding domain and contains an RNP consensus sequence. Antisera from approximately 8% of humans with the autoimmune disease scleroderma recognize fibrillarin. [provided by RefSeq, Jul 2008]
Comments
| Disease | Target Count |
|---|---|
| Autoimmune Diseases | 33 |
| Mammary Neoplasms | 410 |
| Disease | Target Count | P-value |
|---|---|---|
| sonic hedgehog group medulloblastoma | 1482 | 2.45748335122785E-10 |
| atypical teratoid/rhabdoid tumor | 1095 | 2.58602434642568E-9 |
| acute quadriplegic myopathy | 1157 | 1.31485427314438E-8 |
| glioblastoma | 5572 | 1.18536914082982E-7 |
| pediatric high grade glioma | 2712 | 3.7482775917467E-7 |
| medulloblastoma, large-cell | 6234 | 3.75788560310746E-6 |
| lung cancer | 4473 | 7.19074599431449E-6 |
| invasive ductal carcinoma | 2950 | 2.59878382936272E-5 |
| primitive neuroectodermal tumor | 3031 | 1.8907629194343E-4 |
| Multiple myeloma | 1328 | 2.56079231132267E-4 |
| intraductal papillary-mucinous neoplasm (IPMN) | 3289 | 0.00115739126068045 |
| cutaneous lupus erythematosus | 1056 | 0.00199896055089447 |
| osteosarcoma | 7933 | 0.00218324555338565 |
| Waldenstrons macroglobulinemia | 765 | 0.00719493529732331 |
| Disease | Target Count | Z-score | Confidence |
|---|---|---|---|
| Liver Cirrhosis | 101 | 5.566 | 2.8 |
| Hepatitis C | 90 | 5.119 | 2.6 |
| Hepatitis B | 103 | 5.097 | 2.5 |
| Fatty liver disease | 86 | 4.741 | 2.4 |
| Systemic scleroderma | 66 | 4.404 | 2.2 |
| Hepatitis | 61 | 4.34 | 2.2 |
| Vascular disease | 281 | 3.937 | 2.0 |
| Disseminated intravascular coagulation | 32 | 3.136 | 1.6 |
| Cancer | 2346 | 3.124 | 1.6 |
| Disease | log2 FC | p |
|---|---|---|
| Waldenstrons macroglobulinemia | 1.113 | 0.007 |
| Multiple myeloma | 2.218 | 0.000 |
| cutaneous lupus erythematosus | -1.400 | 0.002 |
| osteosarcoma | -1.370 | 0.002 |
| glioblastoma | 1.100 | 0.000 |
| sonic hedgehog group medulloblastoma | 2.300 | 0.000 |
| atypical teratoid/rhabdoid tumor | 1.700 | 0.000 |
| medulloblastoma, large-cell | 2.400 | 0.000 |
| primitive neuroectodermal tumor | 1.300 | 0.000 |
| acute quadriplegic myopathy | 1.199 | 0.000 |
| intraductal papillary-mucinous neoplasm ... | -1.300 | 0.001 |
| lung cancer | 2.200 | 0.000 |
| pediatric high grade glioma | 1.200 | 0.000 |
| invasive ductal carcinoma | -1.353 | 0.000 |
| PMID | Text |
|---|---|
| 24029231 | FBL overexpression contributes to tumorigenesis and is associated with poor survival in patients with breast cancer. |
| 22909121 | NS1 protein of the human H3N2 virus interacts primarily via the C-terminal NLS2/NoLS and to a minor extent via the N-terminal NLS1 with the main nucleolar proteins, nucleolin, B23 and fibrillarin. |
| 21536856 | p32 is a new rRNA maturation factor involved in the remodeling from pre-90S particles to pre-40S and pre-60S particles that requires the exchange of FBL for Nop52. |
| 20628086 | Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator) |
| 19913121 | Observational study of gene-disease association. (HuGE Navigator) |
| 19331828 | Data demonstrate that fibrillarin and Nop56 directly interact in vivo, and that this interaction is indispensable for the association of both proteins with the box C/D snoRNPs. |
| 18292223 | Evidence that BIG1 and nucleolin, but not fibrillarin, can be present with p62 at the nuclear envelope confirms the presence of BIG1 and nucleolin in dynamic molecular complexes that change in composition while moving through nuclei |
| 17603021 | Our data suggest that fibrillarin would play a critical role in the maintenance of nuclear shape and cellular growth. |
| 17461797 | Increased levels of exogenous BRAG2 in nucleoli result in redistribution of FBL to the nucleolar periphery. |
MKPGFSPRGGGFGGRGGFGDRGGRGGRGGFGGGRGRGGGFRGRGRGGGGGGGGGGGGGRGGGGFHSGGNR 1 - 70 GRGRGGKRGNQSGKNVMVEPHRHEGVFICRGKEDALVTKNLVPGESVYGEKRVSISEGDDKIEYRAWNPF 71 - 140 RSKLAAAILGGVDQIHIKPGAKVLYLGAASGTTVSHVSDIVGPDGLVYAVEFSHRSGRDLINLAKKRTNI 141 - 210 IPVIEDARHPHKYRMLIAMVDVIFADVAQPDQTRIVALNAHTFLRNGGHFVISIKANCIDSTASAEAVFA 211 - 280 SEVKKMQQENMKPQEQLTLEPYERDHAVVVGVYRPPPKVKN 281 - 321 //
| PMID | Year | Title |
|---|---|---|
| 24352239 | 2014 | Glutamine methylation in histone H2A is an RNA-polymerase-I-dedicated modification. |
| 24275569 | 2014 | An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. |
| 24029231 | 2013 | p53 acts as a safeguard of translational control by regulating fibrillarin and rRNA methylation in cancer. |
| 23186163 | 2013 | Toward a comprehensive characterization of a human cancer cell phosphoproteome. |
| 22916032 | 2012 | NOL11, implicated in the pathogenesis of North American Indian childhood cirrhosis, is required for pre-rRNA transcription and processing. |
| 22909121 | 2012 | Influenza A H3N2 subtype virus NS1 protein targets into the nucleus and binds primarily via its C-terminal NLS2/NoLS to nucleolin and fibrillarin. |
| 22814378 | 2012 | N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB. |
| 22720776 | 2012 | PHF6 interacts with the nucleosome remodeling and deacetylation (NuRD) complex. |
| 22681889 | 2012 | The mRNA-bound proteome and its global occupancy profile on protein-coding transcripts. |
| 22658674 | 2012 | Insights into RNA biology from an atlas of mammalian mRNA-binding proteins. |
| More... |
