Myelin PLP,髓***酯髓鞘蛋白1抗体

产品编号HZ-11093R
英文名称Myelin PLP
中文名称髓***酯髓鞘蛋白1抗体
别 名Lipophilin; HLD1; Lipophilin; Major myelin proteolipid; Major myelin proteolipid protein; MMPL; Myelin proteolipid protein; MYPR_HUMAN; PLP 1; PLP; PLP/DM20; PLP1; PLP1 protein; PMD; Proteolipid protein 1 (Pelizaeus Merzbacher disease spastic paraplegia 2 uncomplicated); myelin proteolipid protein or lipophilin; Proteolipid protein 1; SPG 2; SPG2.
说 明 书0.1ml 0.2ml
研究领域神经生物学 细胞粘附分子 细胞类型标志物 细胞膜蛋白
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Monkey,
Myelin PLP,髓***酯髓鞘蛋白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.
分 子 量30kDa
细胞定位细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Myelin proteolipid protein
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.
Myelin PLP,髓***酯髓鞘蛋白1抗体PubMedPubMed
产品介绍background:
PLP is a major constituent of myelin. The two isoforms of the myelin proteolipid protein, PLP and DM20, are very hydrophobic integral membrane proteins that account for about half of the protein content of adult CNS myelin. A mutation in the gene which encodes PLP is linked to Pelizaeus-Merzbacher disease (PMD), a chronic infantile type of diffuse cerebral sclerosis. The gene which encodes PLP maps to human chromosome Xq22. The glycoprotein zero (also designated P-zero or myelin peripheral protein) is the primary structural protein of peripheral myelin, and accounts for more than 50% of the protein present in the peripheral nerve sheath. Zero is an integral membrane glycoprotein. Expression of zero is restricted to Schwann cells. The gene which encodes zero maps to human chromosome 1q22. PMP22 (peripheral myelin protein 22) is a growth-regulated membrane protein which is expressed by Schwann cells and is localized primarily in compact peripheral nervous system myelin. The gene which encodes PMP22 maps to human chromosome 17p11.2.

Function:
This is the major myelin protein from the central nervous system. It plays an important role in the formation or maintenance of the multilamellar structure of myelin.

Subunit:
Belongs to the myelin proteolipid protein family.

Subcellular Location:
Membrane; Multi-pass membrane protein.

Myelin PLP,髓***酯髓鞘蛋白1抗体DISEASE:
Defects in PLP1 are the cause of leukodystrophy hypomyelinating type 1 (HLD1) [MIM:312080]; also known as Pelizaeus-Merzbacher disease. HLD1 is an X-linked recessive dysmyelinating disorder of the central nervous system in which myelin is not formed properly. It is characterized clinically by nystagmus, spastic quadriplegia, ataxia, and developmental delay.
Defects in PLP1 are the cause of spastic paraplegia X-linked type 2 (SPG2) [MIM:312920]. SPG2 is characterized by spastic gait and hyperreflexia. In some patients, complicating features include nystagmus, dysarthria, sensory disturbance, mental retardation, optic atrophy.

Similarity:
Belongs to the myelin proteolipid protein family.

Gene ID:
5354

Database links:
Entrez Gene: 281410 Cow
Entrez Gene: 481002 Dog
Entrez Gene: 5354 Human
Entrez Gene: 18823 Mouse
Entrez Gene: 24943 Rat
Omim: 300401 Human
SwissProt: P04116 Cow
SwissProt: P23294 Dog
SwissProt: P60201 Human
SwissProt: P60202 Mouse
SwissProt: P60203 Rat
Unigene: 49691 Cow
Unigene: 1787 Human
Unigene: 1268 Mouse
Unigene: 4550 Rat

Myelin PLP,髓***酯髓鞘蛋白1抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品图片

Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;  Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;  Incubation: Anti-Myelin PLP Polyclonal Antibody, Unconjugated(HZ-11093R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Myelin PLP) Polyclonal Antibody, Unconjugated (HZ-11093R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.