LRSAM1 Antibody

說明書

中文名稱：

LRSAM1兔多克隆抗體
貨號：

CSB-PA751010ESR2HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human skin tissue using CSB-PA751010ESR2HU at dilution of 1:100
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) LRSAM1 Polyclonal antibody
Uniprot No.：

Q6UWE0
基因名：

LRSAM1
別名：

CMT2P antibody; E3 ubiquitin protein ligase LRSAM1 antibody; E3 ubiquitin-protein ligase LRSAM1 antibody; EC 6.3.2.- antibody; FLJ31641 antibody; hTAL antibody; Leucine rich repeat and sterile alpha motif containing 1 antibody; Leucine rich repeat and sterile alpha motif containing protein 1 antibody; Leucine-rich repeat and sterile alpha motif-containing protein 1 antibody; LRSAM1 antibody; LRSM1_HUMAN antibody; OTTHUMP00000022174 antibody; OTTHUMP00000022175 antibody; RIFLE antibody; TAL antibody; Tsg101 associated ligase antibody; Tsg101-associated ligase antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human E3 ubiquitin-protein ligase LRSAM1 protein (1-290AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

E3 ubiquitin-protein ligase that mediates monoubiquitination of TSG101 at multiple sites, leading to inactivate the ability of TSG101 to sort endocytic (EGF receptors) and exocytic (HIV-1 viral proteins) cargos. Bacterial recognition protein that defends the cytoplasm from invasive pathogens. Localizes to several intracellular bacterial pathogens and generates the bacteria-associated ubiquitin signal leading to autophagy-mediated intracellular bacteria degradation (xenophagy).
基因功能參考文獻：
1. We identified a novel LRSAM1 missense mutation (c.2120C > T, p.Pro707Leu) mapping to the RING domain. The identified missense mutation, as well as of another recently reported pathogenic missense mutation (c.2081G > A, p.Cys694Tyr), revealed that in vitro ubiquitylation activity was largely abrogated. PMID: 28335037
2. findings suggest that the mutant LRSAM1 may aberrantly affect the formation of transcription machinery. PMID: 27615052
3. findings demonstrate that the isolated genetic entity Charcot-Marie-Tooth type 2G is caused by a missense mutation in LRSAM1. PMID: 27686364
4. LRSAM1 exhibited self-association in vitro and in vivo. The study found the self-association of LRSAM1 promotes intermolecular ubiquitination and proved a potential N-terminal ubiquitination. PMID: 28189685
5. disruption of the C-terminal RING domain confers dominant negative properties to LRSAM1 PMID: 24894446
6. Our data further confirms that LRSAM1 mutations are associated with CMT2 of AD inheritance. PMID: 22781092
7. Authors identify LRSAM1 as the E3 ligase responsible for anti-Salmonella autophagy-associated ubiquitination. PMID: 23245322
8. homozygous mutation in LRSAM1 was proposed as a strong candidate for the disease in a family with recessive axonal polyneuropathy PMID: 22012984
9. LRSAM1 as a component of the antibacterial autophagic response. PMID: 20616063
10. LRSAM1 is a strong candidate for the causal gene for the Charcot-Marie-Tooth disease. PMID: 20865121
11. Results suggest that RIFLE represents a novel signaling protein that mediates components of the Wnt/wingless signaling pathway and cell adhesion in PC12 cells [RIFLE protein]. PMID: 14635195
12. Tal regulates a Tsg101-associated complex responsible for the sorting of cargo into cytoplasm-containing vesicles that bud at the multivesicular body and at the plasma membrane PMID: 15256501
13. Tal polyubiquitinates lysine residues in the C-terminus of uncomplexed Tsg101, resulting in proteasomal degradation. PMID: 18077552
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相關疾病：

Charcot-Marie-Tooth disease 2P (CMT2P)
亞細胞定位：

Cytoplasm.
組織特異性：

Highly expressed in adult spinal cord motoneurons as well as in fetal spinal cord and muscle tissue.
數據庫鏈接：

HGNC: 25135

OMIM: 610933

KEGG: hsa:90678

STRING: 9606.ENSP00000300417

UniGene: Hs.495188

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