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Rabbit Anti-IP3 receptor/RBITC Conjugated antibody (bs-4242R-RBITC)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-4242R-RBITC
英文名称1 Rabbit Anti-IP3 receptor/RBITC Conjugated antibody
中文名称 罗丹明(RBITC)标记的5-三磷酸肌醇受体1抗体
别    名 Type 1 inositol 1 4 5 trisphosphate receptor; Type 1 inositol 1; Type 1 InsP3 receptor.; 5-trisphosphate receptor; 5-trisphosphate receptor type 1; DKFZp313E1334; DKFZp313N1434; inositol 1 4 5 triphosphate receptor type 1; Inositol 1 4 5 trisphosphate Receptor Type 1; Inositol 1; Inositol 145-trisphosphate Receptor Type 1; InsP3R1; IP3; IP3 receptor; IP3 receptor isoform 1; IP3R 1; IP3R; IP3R1; ITPR 1; Itpr1; ITPR1_HUMAN; SCA15; SCA16; SCA29; ACV; CLA4; INSP3R1.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  免疫学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 303kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ITPR1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
This gene encodes an intracellular receptor for inositol 1,4,5-trisphosphate. Upon stimulation by inositol 1,4,5-trisphosphate, this receptor mediates calcium release from the endoplasmic reticulum. Mutations in this gene cause spinocerebellar ataxia type 15, a disease associated with an heterogeneous group of cerebellar disorders. Multiple transcript variants have been identified for this gene. [provided by RefSeq, Nov 2009].

Function:
Intracellular channel that mediates calcium release from the endoplasmic reticulum following stimulation by inositol 1,4,5-trisphosphate.

Subunit:
Homotetramer. Interacts with TRPC4. The PPXXF motif binds HOM1, HOM2 and HOM3. Interacts with RYR1, RYR2, ITPR1, SHANK1 and SHANK3. Interacts with ERP44 in a pH-, redox state- and calcium-dependent manner which results in the inhibition the calcium channel activity. The strength of this interaction inversely correlates with calcium concentration. Part of cGMP kinase signaling complex at least composed of ACTA2/alpha-actin, CNN1/calponin H1, PLN/phospholamban, PRKG1 and ITPR1. Interacts with AHCYL1 (By similarity). Interacts with MRVI1 and CABP1 (via N-terminus).

Subcellular Location:
Endoplasmic reticulum membrane.

Tissue Specificity:
Widely expressed.

Post-translational modifications:
Phosphorylated by cAMP kinase.
Phosphorylation prevents the ligand-induced opening of the calcium channels.
Phosphorylated on tyrosine residues.

DISEASE:
Defects in ITPR1 are the cause of spinocerebellar ataxia type 15 (SCA15) (SCA15) [MIM:606658]. Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA15 is an autosomal dominant cerebellar ataxia (ADCA). It is very slow progressing form with a wide range of onset, ranging from childhood to adult. Most patients remain ambulatory.

Similarity:
Belongs to the InsP3 receptor family.
Contains 5 MIR domains.

Database links:

Entrez Gene: 476548 Dog

Entrez Gene: 3708 Human

Entrez Gene: 16438 Mouse

Entrez Gene: 25262 Rat

Omim: 147265 Human

SwissProt: Q14643 Human

SwissProt: P11881 Mouse

SwissProt: P29994 Rat

Unigene: 567295 Human

Unigene: 715765 Human

Unigene: 227912 Mouse

Unigene: 2135 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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