英文名称 RSPH4A
中文名称 Kartagener综合征相关蛋白RSHL3抗体
别 名 CILD11; dJ412I7.1; Radial spoke head protein 4 homolog A; Radial spoke head-like protein 3; RSH4A_HUMAN; RSHL3; Rsph4a; RSPH6B; A230081C05.
Kartagener综合征相关蛋白RSHL3抗体
说 明 书 0.1ml 0.2ml
研究领域 细胞生物 发育生物学 神经生物学 信号转导 细胞骨架 细胞外基质
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
产品应用 WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 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 81kDa
细胞定位 细胞浆
性 状 Lyophilized or Liquid
浓 度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human RSPH4A/RSHL3 (435-482aa)
亚 型 IgG
纯化方法 affinity purified by Protein A
储 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
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.
Kartagener综合征相关蛋白RSHL3抗体产品介绍 background:
RSHL3 is predicted to be a component of the radial spoke head based on homology with proteins in the biflagellate alga Chlamydomonas reinhardtii and other ciliates. RSHL3 (radial spoke head-like protein 3), also known as radial spoke head protein 4 homolog A, is a 716 amino acid protein that belongs to the flagellar radial spoke RSP4/6 family. Mutations in the RSHL3 gene cause primary ciliary dyskinesia 1, a disease arising from dysmotility of motile cilia and sperm. Existing as three alternatively spliced isoforms, the RSHL3 gene contains 6 exons, is conserved in chimpanzee, dog, cow, mouse, rat, chicken, zebrafish, fruit fly and P.falciparum, and maps to human chromosome 6q22.1.
Function:
Probable component of the axonemal radial spoke head. Radial spokes are regularly spaced along cilia, sperm and flagella axonemes. They consist of a thin stalk which is attached to a subfiber of the outer doublet microtubule, and a bulbous head which is attached to the stalk and appears to interact with the projections from the central pair of microtubules.
Subcellular Location:
Cytoplasm; cytoskeleton; cilium axoneme. Radial spoke.
Tissue Specificity:
Defects in RSPH4A are the cause of primary ciliary dyskinesia type 11 (CILD11) [MIM:612649]. CILD is an autosomal recessive disorder characterized by axonemal abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit situs inversus, due to dysfunction of monocilia at the embryonic node and randomization of left-right body asymmetry. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.
DISEASE:
Defects in RSPH4A are the cause of primary ciliary dyskinesia type 11 (CILD11) [MIM:612649]. CILD is an autosomal recessive disorder characterized by axonemal abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit situs inversus, due to dysfunction of monocilia at the embryonic node and randomization of left-right body asymmetry. Primary ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome.
Similarity:
Belongs to the flagellar radial spoke RSP4/6 family.
Database links:
Entrez Gene: 345895 Human
Omim: 612647 Human
SwissProt: Q5TD94 Human
Unigene: 160380 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Kartagener综合征相关蛋白RSHL3抗体
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hz-2100P EPEC/E.coli(enteropathogenic E.coli,EPEC) 普通大肠埃希菌菌体蛋白(非致病性)
hz-1763P EphA1 R(Ephrin A1 Receptor) EphA1-R受体抗原
hz-1424P EPOR peptide 红细胞生成素受体抗原
hz-1522P phospho-ERK1/MAPK-1/2(pThr183 + pTyr185) 磷酸化丝裂原活化蛋白激酶1/2抗原
hz-0996P ERK(Extracellular signal-regulated kinase) 丝裂原活化蛋白激酶抗原
hz-1020P MAPK1/ERK3 (Mitogen-activated protein kinase 3; P44MAPK) 丝裂原活化蛋白激酶3抗原
hz-1319P MAPK4(Mitogen-activated protein kinase 4) 丝裂原活化蛋白激酶4抗原
hz-0894P ER- Alpha(phospho-Ser167)peptide 人磷酸化雌激素受体α多肽抗原
hz-1643P ER- Alpha(phospho-Tyr537)peptide 磷酸化雌激素受体α抗原
hz-0253P ER- Alpha /ER alpha peptide 雌激素受体α抗原
hz-2098P ER- Alpha/ER alpha peptide 雌激素受体α抗原
hz-0255P ER- Beta (Estrogen receptor-beta) peptide 雌激素受体β抗原
hz-1285P ets1/E26 (E-twenty six 1) Ets转录因子家族ets1抗原
hz-0852P EV71(Human enterovirus 71)VP1 肠道病毒71型/手足口病病毒抗原
hz-0983P EV71(Human enterovirus 71)VP1(CT) 肠道病毒71型/手足口病病毒抗原(C端)
hz-2221P ATEXPA10 peptide 植物扩张蛋白ATEXPA10(拟南芥)抗原
hz-1343P Ezrin/Radixin 埃兹蛋白(多肽)
hz-0949P F1 operon positive regulatory protein(NT) 鼠疫耶尔森氏菌F1荚膜蛋白(N端多肽)
hz-0950P F1 operon positive regulatory protein(CT) 鼠疫耶尔森氏菌F1荚膜蛋白(C端多肽)
hz-2135P FABP(brain) (Fatty acid-binding protein brain) 脑型脂肪酸结合蛋白抗原
hz-0511P FADD (Fas-associated protein with death domain) Fas死亡结构域相关蛋白抗原
hz-1340P FAK(Focaladhesin kinase) 粘着斑激酶抗原
hz-1639P phospho-FAK(pTyr577) 磷酸化粘着斑激酶抗原
hz-1642P phospho-FAK(pSer732)(phospho-Focal adhesion kinase) 磷酸化粘着斑激酶抗原
hz-1765P FAST kinase(Fas-activated serine/threonine kinase) Fas活化的丝/苏氨酸激酶抗原
hz-1157P FBN1 (fibrillin 1) 原纤维蛋白1抗原
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hz-1257P FGF5 peptide 纤维母细胞生长因子5抗原
hz-0809P Fibulin 1 衰老关键蛋白抗原
hz-1462P FLG(filaggrin)peptide 丝聚蛋白/中间丝蛋白抗原
hz-1170P FOS B FosB抗原