IP3 receptor Recombinant Rabbit mAb (一抗) - IHC-P,IHC-F,IF,Flow-Cyt,ICC/IF | Bioss

bsm-61555R

重组兔单抗、mIHC精品抗体

IP3 receptor Recombinant Rabbit mAb

5-三磷酸肌醇受体1重组兔单抗

ACV; CLA4; INSP3R1; IP3R; IP3R1; PPP1R94; SCA15; SCA16; SCA29; D6Pas2; Gm10429; IP3R 1; InsP3R; Itpr-1; P400; Pcp-1; Pcp1; opt; wblo; I145TR; ITPR1_HUMAN; ITPR1; IP3 receptor isoform 1 (IP3R 1 | InsP3R1); Inositol 1,4,5 trisphosphate receptor; Inositol 1,4,5-trisphosphate receptor type 1; Type 1 inositol 1,4,5-trisphosphate receptor (Type 1 InsP3 receptor); ITPR1_MOUSE; Inositol 1,4,5-trisphosphate-binding protein P400; Protein PCD-6; Purkinje cell protein 1; Pcd6; ITPR1_RAT; Inositol 1,4,5-trisphosphate receptor (IP-3-R); spinocerebellar ataxia 15; spinocerebellar ataxia 16; spinocerebellar ataxia 29; inositol 1,4,5-trisphosphate receptor, type 1; protein phosphatase 1, regulatory subunit 94

Rabbit

A synthesized peptide derived from human ITPR1: 2200-2300

IgG

Liquid

affinity purified by Protein A

Recombinant

13E1

314 kDa

314 kDa

10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.

Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.

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

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.

ITPR1

Inositol 1, 4, 5-trisphosphate receptor type 1

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).

Endoplasmic reticulum membrane.

Widely expressed.

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

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.

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

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