产品货号 : mlR18686
英文名称 : MASS1/GPR98
中文名称 : G蛋白偶联受体98抗体
别 名 : DKFZp761P0710; FEB 4; FEB4; G protein coupled receptor 98; G-protein coupled receptor 98; GPR 98; GPR98; GPR98_HUMAN; KIAA0686; MASS 1; Monogenic audiogenic seizure susceptibility 1 homolog; Monogenic audiogenic seizure susceptibility protein 1 homolog; USH 2B; USH 2C; USH2B; USH2C; Usher syndrome 2C; Usher syndrome type 2C protein; Usher syndrome type-2C protein; Very large G protein coupled receptor; Very large G protein coupled receptor 1; Very large G-protein coupled receptor 1; VLGR 1; VLGR 1b; VLGR1; VLGR1b.
研究领域 : 细胞生物 神经生物学
抗体来源 : Rabbit
克隆类型 : Polyclonal
交叉反应 : Human, Mouse, Rat, Cow, Horse, Sheep,
产品应用 : ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 : 693kDa
细胞定位 : 细胞膜
性 状 : Lyophilized or Liquid
浓 度 : 1mg/ml
免 疫 原 : KLH conjugated synthetic peptide derived from human MASS1/GPR98:2451-2550/6306 <Extracellular>
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.
PubMed : PubMed
产品介绍 MASS1 (for monogenic audiogenic seizure susceptibility 1) is one of the largest known GPCRs and is therefore referred to as Very Large G protein-coupled receptor 1 (VLGR1) (1,2). MASS1 is a large, calcium-binding GPCR expressed in the central nervous system and the eye (2,3). MASS1 has a large ectodomain containing multiple calcium exchanger beta repeats that resemble regulatory domains of sodium-calcium exchanger proteins (3). The human MASS1 gene maps to chromosome 5q14 and encodes a 1967 amino acid protein (1,2,4). The MASS1 gene has been linked to the autosomal recessive inheritance of general epilepsy in Frings mice that have seizures in response to loud noises (5).
Function:
Receptor that may have an important role in the development of the central nervous system.
Subcellular Location:
Cell membrane.
Tissue Specificity:
Expressed at low levels in adult tissues.
DISEASE:
Defects in GPR98 are the cause of Usher syndrome type 2C (USH2C) [MIM:605472]. USH is a genetically heterogeneous condition characterized by the association of retinitis pigmentosa with sensorineural deafness. Age at onset and differences in auditory and vestibular function distinguish Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2) and Usher syndrome type 3 (USH3). USH2 is characterized by congenital mild hearing impairment with normal vestibular responses.
Defects in GPR98 may be a cause of familial febrile convulsions type 4 (FEB4) [MIM:604352]; also known as familial febrile seizures 4. Febrile convulsions are seizures associated with febrile episodes in childhood without any evidence of intracranial infection or defined pathologic or traumatic cause. It is a common condition, affecting 2-5% of children aged 3 months to 5 years. The majority are simple febrile seizures (generally defined as generalized onset, single seizures with a duration of less than 30 minutes). Complex febrile seizures are characterized by focal onset, duration greater than 30 minutes, and/or more than one seizure in a 24 hour period. The likelihood of developing epilepsy following simple febrile seizures is low. Complex febrile seizures are associated with a moderately increased incidence of epilepsy.
Similarity:
Belongs to the G-protein coupled receptor 2 family.
LN-TM7 subfamily
. Contains 35 Calx-beta domains.
Contains 6 EAR repeats.
Contains 1 GPS domain.
SWISS:
Q8WXG9
Gene ID:
84059
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
