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产品信息
PA5-88585
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Recombinant fusion protein containing a sequence corresponding to amino acids 364-631 of human PEX5 (NP_0003102)
偶联物
形式
Liquid
浓度
2.13 mg/mL
纯化类型
Affinity Chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.01% thimerosal
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
RRID
AB_2805015
产品详细信息
Immunogen sequence: WQYLGTTQAE NEQELLAISA LRRCLELKPD NQTALMALAV SFTNESLQRQ ACETLRDWLR YTPAYAHLVT PAEEGAGGAG LGPSKRILGS LLSDSLFLEV KELFLAAVRL DPTSIDPDVQ CGLGVLFNLS GEYDKAVDCF TAALSVRPND YLLWNKLGAT LANGNQSEEA VAAYRRALEL QPGYIRSRYN LGISCINLGA HREAVEHFLE ALNMQRKSRG PRGEGGAMSE NIWSTLRLAL SMLGQSDAYG AADARDLSTL LTMFGLPQ; Positive Samples: U-251MG, HepG2, 293T, HeLa, Mouse brain, Mouse kidney, Mouse liver; Cellular Location: Cytoplasm, Peripheral membrane protein, Peroxisome membrane
靶标信息
The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: peroxin 5; Peroxin-5; peroxisomal biogenesis factor 5; Peroxisomal C-terminal targeting signal import receptor; peroxisomal targeting signal 1 (SKL type) receptor; Peroxisomal targeting signal 1 receptor; peroxisomal targeting signal import receptor; peroxisomal targeting signal receptor 1; peroxisome biogenesis factor 5; Peroxisome receptor 1; PTS1 BP; PTS1 receptor; PTS1-BP; PXR1P
基因别名: AW212715; ESTM1; PBD2A; PBD2B; PEX5; Pex5_predicted; PTS1-BP; PTS1R; PXR1; RCDP5; X83306
UniProt ID: (Human) P50542, (Mouse) O09012, (Rat) Q2M2R8
Entrez Gene ID: (Human) 5830, (Mouse) 19305, (Rat) 312703
protein import into peroxisome matrix
protein import into peroxisome matrix, docking
protein import into peroxisome membrane
negative regulation of protein homotetramerization
very long-chain fatty acid metabolic process
neuron migration
protein targeting to peroxisome
fatty acid beta-oxidation
transport
mitochondrion organization
mitochondrial membrane organization
endoplasmic reticulum organization
peroxisome organization
isoprenoid biosynthetic process
protein transport
protein import into peroxisome matrix, translocation
cerebral cortex cell migration
cerebral cortex neuron differentiation
positive regulation of multicellular organism growth
cellular lipid metabolic process
cell development
neuromuscular process
protein tetramerization
biological_process
