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Product Name FCP1 Chinese Name FCP1蛋白抗体 Alias CCFDN; CTD (carboxy-terminal domain, RNA polymerase II, polypeptide A) phosphatase, subunit 1; CTD of POLR2A, phosphatase of, subunit 1; CTDP1; CTDP1_HUMAN; EC 3.1.3.16; RNA polymerase II subunit A C terminal domain phosphatase; RNA polymerase II subunit A C-terminal domain phosphatase; Serine phosphatase FCP1a; TFIIF associating CTD phosphatase; TFIIF-associating CTD phosphatase 1; TFIIF-associating CTD phosphatase; Transcription factor IIF-associating CTD phosphatase 1. Research Area Cell biology Neurobiology Signal transduction Kinases and Phosphatases Epigenetics Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, Rat, (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Sheep, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 104kDa Cellular localization The nucleus Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human FCP1: 201-300/961 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail This gene encodes a protein which interacts with the carboxy-terminus of the RAP74 subunit of transcription initiation factor TFIIF, and functions as a phosphatase that processively dephosphorylates the C-terminus of POLR2A (a subunit of RNA polymerase II), making it available for initiation of gene expression. Mutations in this gene are associated with congenital cataracts, facial dysmorphism and neuropathy syndrome (CCFDN). Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Feb 2011]
Function:
Processively dephosphorylates 'Ser-2' and 'Ser-5' of the heptad repeats YSPTSPS in the C-terminal domain of the largest RNA polymerase II subunit. This promotes the activity of RNA polymerase II.
Subcellular Location:
Nucleus.
Tissue Specificity:
Ubiquitously expressed. Isoform 3 is expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney and placenta.
Post-translational modifications:
Phosphorylated. In the presence of TFIIF, the phosphorylated form has an increased CTD phosphatase activity. The phosphorylation is required for the physical interaction with GTF2F1.
DISEASE:
Defects in CTDP1 are a cause of congenital cataracts facial dysmorphism and neuropathy syndrome (CCFDN) [MIM:604168]. CCFDN is an autosomal recessive developmental disorder that occurs in an endogamous group of Vlax Roma (Gypsies). The syndrome is characterized by a complex clinical phenotype with seemingly unrelated features involving multiple organs and systems. Developmental abnormalities include congenital cataracts and microcorneae, hypomyelination of the peripheral nervous system, impaired physical growth, delayed early motor and intellectual development, facial dysmorphism and hypogonadism. Central nervous system involvement, with cerebral and spinal cord atrophy, may be the result of disrupted development with superimposed degenerative changes. Affected individuals are prone to severe rhabdomyolysis after viral infections and to serious complications related to general anesthesia (such as pulmonary edema and epileptic seizures).
Similarity:
Contains 1 BRCT domain.
Contains 1 FCP1 homology domain.
SWISS:
Q9Y5B0
Gene ID:
9150
Database links:Entrez Gene: 9150 Human
Entrez Gene: 67655 Mouse
Entrez Gene: 447713 Xenopus laevis
Entrez Gene: 442930 Zebrafish
Omim: 604927 Human
SwissProt: Q9Y5B0 Human
SwissProt: Q7TSG2 Mouse
Unigene: 465490 Human
Unigene: 312893 Mouse
Unigene: 137952 Rat
Unigene: 62700 Xenopus laevis
Unigene: 133831 Zebrafish
Product Picture Sample:
Heart(Mouse) Lysate at 40 ug
Primary: Anti- FCP1 (SL16063R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 104 kD
Observed band size: 104 kD
Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (FCP1) Polyclonal Antibody, Unconjugated (SL16063R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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