Rabbit Polyclonal Antibody to CEP104

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别名:GlyBP; ROC22; JBTS25; CFAP256; KIAA0562
应用:IHC
反应种属:Human
规格：50μl/100μl

Description
This gene encodes a centrosomal protein required for ciliogenesis and for ciliary tip structural integrity. The mammalian protein contains three amino-terminal hydrophobic domains, two glycosylation sites, four cysteine-rich motifs, and two regions with homology to the glutamate receptor ionotropic, NMDA 1 protein. During ciliogenesis, the encoded protein translocates from the distal tips of the centrioles to the tip of the elongating cilium. Knockdown of the protein in human retinal pigment cells results in severe defects in ciliogenesis with structural deformities at the ciliary tips. Allelic variants of this gene are associated with the autosomal-recessive disorder Joubert syndrome, which is characterized by a distinctive mid-hindbrain and cerebellar malformation, oculomotor apraxia, irregular breathing, developmental delay, and ataxia.

Description

This gene encodes a centrosomal protein required for ciliogenesis and for ciliary tip structural integrity. The mammalian protein contains three amino-terminal hydrophobic domains, two glycosylation sites, four cysteine-rich motifs, and two regions with homology to the glutamate receptor ionotropic, NMDA 1 protein. During ciliogenesis, the encoded protein translocates from the distal tips of the centrioles to the tip of the elongating cilium. Knockdown of the protein in human retinal pigment cells results in severe defects in ciliogenesis with structural deformities at the ciliary tips. Allelic variants of this gene are associated with the autosomal-recessive disorder Joubert syndrome, which is characterized by a distinctive mid-hindbrain and cerebellar malformation, oculomotor apraxia, irregular breathing, developmental delay, and ataxia.

Specification
Aliases	GlyBP; ROC22; JBTS25; CFAP256; KIAA0562
Swissprot	O60308
Host/Isotype	Rabbit IgG
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human
Immunogen	Synthetic peptide of human CEP104
Formulation	pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Application
IHC	1/25-1/100
ELISA	1/5000-1/10000

	The image is immunohistochemistry of paraffin-embedded Human colorectal cancer tissue using P06180(CEP104 Antibody) at dilution 1/25. (Original magnification: ×200)
	The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P06180(CEP104 Antibody) at dilution 1/25. (Original magnification: ×200)