Product Name	RP1
Chinese Name	视网膜色素变性蛋白1抗体
Alias	DCDC4A; ORP1; Oxygen-regulated protein 1; Retinitis pigmentosa 1 protein; Retinitis pigmentosa RP1 protein; RP1; RP1_HUMAN.
Research Area	Developmental biology  Neurobiology  Cell adhesion molecule  Cytoskeleton
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Dog, Horse, Rabbit, )
Applications	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	241kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human RP1/DCDC4A: 451-550/2156
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	Retinitis pigmentosa 1 is a novel 2,156 amino acid oxygen-regulated photoreceptor specific to retina. Originally named ORP1 (for 'oxygen-regulated protein-1'), the expression of retinitis pigmentosa 1 has been found to be regulated by oxygen levels in the retina. Mutation of the retinitis pigmentosa 1 gene causes dominant retinitis pigmentosa which leads to degeneration of retinal photoreceptor cells and symptoms such as night vision blindness and deficits in the midperipheral visual field. Retinitis pigmentosa 1 may assist in differentiation of photoreceptor cells and has been identified in the cilia of photoreceptors, possibly aiding in both cilial structure and protein transport between inner and outer segments of photoreceptors. Retinitis pigmentosa 1 contains two doublecortin domains and is encoded by a gene which maps to human chromosome 8q11-q13. Function: Microtubule-associated protein regulating the stability and length of the microtubule-based axoneme of photoreceptors. Required for the differentiation of photoreceptor cells, it plays a role in the organization of the outer segment of rod and cone photoreceptors ensuring the correct orientation and higher order stacking of outer segment disks along the photoreceptor axoneme. Subunit: Interacts (via the doublecortin domains) with microtubules. Interacts with RP1L1 (By similarity). Interacts with MAK (By similarity). Subcellular Location: Cytoplasm; cytoskeleton; cilium axoneme. Cell projection; cilium; photoreceptor outer segment. Specifically localized in the connecting cilia of rod and cone photoreceptors. Tissue Specificity: Expressed in retina. Not expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney, spleen and pancreas. DISEASE: Defects in RP1 are the cause of retinitis pigmentosa type 1 (RP1) [MIM:180100]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. Similarity: Contains 2 doublecortin domains. SWISS: P56715 Gene ID: 6101 Database links: Entrez Gene: 6101 Human Omim: 603937 Human SwissProt: P56715 Human Unigene: 128938 Human

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