N聚糖酶多肽蛋白1抗体
规格：1mg/1ml
英文名： NGLY1
别名： CDG1V; hPNGase; N glycanase 1; NGLY 1; Peptide N(4) (N acetyl beta glucosaminyl asparagine amidase; Peptide:N glycanase; PNG1; PNGase.
分子量： 74kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human NGLY1
交叉反应：Human, Mouse, Rat, Pig, Cow, Rabbit, Cynomolgus Monkey
细胞定位：细胞浆
N聚糖酶多肽蛋白1抗体产品介绍：background: This gene encodes an enzyme that catalyzes hydrolysis of an N(4)-(acetyl-beta-D-glucosaminyl) asparagine residue to N-acetyl-beta-D-glucosaminylamine and a peptide containing an aspartate residue. The encoded enzyme may play a role in the proteasome-mediated degradation of misfolded glycoproteins. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Feb 2009] Function: Specifically deglycosylates the denatured form of N-linked glycoproteins in the cytoplasm and assists their proteasome-mediated degradation. Cleaves the beta-aspartyl-glucosamine (GlcNAc) of the glycan and the amide side chain of Asn, converting Asn to Asp. Prefers proteins containing high-mannose over those bearing complex type oligosaccharides. Can recognize misfolded proteins in the endoplasmic reticulum that are exported to the cytosol to be destroyed and deglycosylate them, while it has no activity toward native proteins. Deglycosylation is a prerequisite for subsequent proteasome-mediated degradation of some, but not all, misfolded glycoproteins. Subunit: Component of a complex required to couple retrotranslocation, ubiquitination and deglycosylation composed of NGLY1, SAKS1, AMFR, VCP and RAD23B. Interacts with the proteasome components RAD23B and PSMC1. Interacts with directly with VCP. Interacts with DERL1, bringing it N聚糖酶多肽蛋白1抗体close to the endoplasmic reticulum membrane. Interacts with SAKS1 Subcellular Location: Cytoplasm DISEASE: A multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Characterized by developmental delay, hypotonia, abnormal involuntary movements and alacrima or poor tear production. Other features include microcephaly, intractable seizures, abnormal eye movements and evidence of liver dysfunction, probably due to cytoplasmic accumulation of storage material in vacuoles. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions.N聚糖酶多肽蛋白1抗体 Similarity: Belongs to the transglutaminase-like superfamily. PNGase family. Contains 1 PAW domain. Contains 1 PUB (PUG) domain. Gene ID: 55768 Database links: Entrez Gene: 55768 Human Entrez Gene: 59007 Mouse Entrez Gene: 361014 Rat Omim: 610661 Human SwissProt: Q4R6F3 Cynomolgus Monkey SwissProt: Q96IV0 Human SwissProt: Q9JI78 Mouse SwissProt: Q5XI55 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
N聚糖酶多肽蛋白1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：细胞生物 神经生物学 转录调节因子 泛素
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid