LMBRD1 Antibody

說(shuō)明書(shū)

中文名稱(chēng)：

LMBRD1兔多克隆抗體
貨號(hào)：

CSB-PA885730LA01HU
規(guī)格：

￥440
圖片：
Western Blot
Positive WB detected in: Mouse liver tissue, Mouse kidney tissue
All lanes: LMBRD1 antibody at 4μg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 62, 45, 54, 22 kDa
Observed band size: 62, 54 kDa

Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA885730LA01HU at dilution of 1:100
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

產(chǎn)品名稱(chēng)：

Rabbit anti-Homo sapiens (Human) LMBRD1 Polyclonal antibody
Uniprot No.：

Q9NUN5
基因名：

LMBRD1
別名：

HDAg-L-interacting protein NESI antibody; LMBD1_HUMAN antibody; LMBR1 domain-containing protein 1 antibody; lmbrd1 antibody; Nuclear export signal-interacting protein antibody; Probable lysosomal cobalamin transporter antibody
宿主：

Rabbit
反應(yīng)種屬：

Human, Mouse
免疫原：

Recombinant Human Probable lysosomal cobalamin transporter protein (6-100AA)
免疫原種屬：

Homo sapiens (Human)

標(biāo)記方式：

Non-conjugated

本頁(yè)面中的產(chǎn)品，LMBRD1 Antibody (CSB-PA885730LA01HU)，的標(biāo)記方式是Non-conjugated。對(duì)于LMBRD1 Antibody，我們還提供其他標(biāo)記。見(jiàn)下表：

標(biāo)記方式	貨號(hào)	產(chǎn)品名稱(chēng)	應(yīng)用
HRP	CSB-PA885730LB01HU	LMBRD1 Antibody, HRP conjugated	ELISA
FITC	CSB-PA885730LC01HU	LMBRD1 Antibody, FITC conjugated
Biotin	CSB-PA885730LD01HU	LMBRD1 Antibody, Biotin conjugated	ELISA

克隆類(lèi)型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

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

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:20-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲(chǔ)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(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

功能：

Lysosomal membrane chaperone required to export cobalamin (vitamin B12) from the lysosome to the cytosol, allowing its conversion to cofactors. Targets ABCD4 transporter from the endoplasmic reticulum to the lysosome. Then forms a complex with lysosomal ABCD4 and cytoplasmic MMACHC to transport cobalamin across the lysosomal membrane. Acts as an adapter protein which plays an important role in mediating and regulating the internalization of the insulin receptor (INSR). Involved in clathrin-mediated endocytosis of INSR via its interaction with adapter protein complex 2. Essential for the initiation of gastrulation and early formation of mesoderm structures during embryogenesis.; (Microbial infection) May play a role in the assembly of hepatitis delta virus (HDV).
基因功能參考文獻(xiàn)：
1. endogenous ABCD4 was localized to both lysosomes and the ER, and its lysosomal localization was disturbed by knockout of LMBRD1 PMID: 27456980
2. Data suggest that ABCD4 lysosomal targeting depends on co-expression of and interaction with LMBRD1; mutations in LMBRD1 and ABCD4 that result in cobalamin metabolism disorders cblF and cblJ (or mutations in ATPase domain) disrupt interactions between LMBRD1 and ABCD4. (LMBRD1 = nuclear export signal-interacting protein; ABCD4 = ATP-binding cassette, sub-family D (ALD), member 4) PMID: 28572511
3. Results propose a model whereby membrane-bound LMBD1 and ABCD4 facilitate the vectorial delivery of lysosomal vitamin B12 to cytoplasmic MMACHC. PMID: 25535791
4. LMBD1 plays an imperative role in mediating and regulating the endocytosis of the IR. PMID: 24078630
5. These data indicate that by forming complexes with lamin A/C and nucleoporins, NESI facilitates the CRM1-independent nuclear export of large hepatitis delta antigen. PMID: 23175358
6. novel mutations in LMBRD1 in three patients PMID: 21303734
7. LMBRD1: the gene for the cblF defect of vitamin B metabolism. PMID: 20446115
8. a LMBRD1 mutation causes the cblF defect of vitamin B(12) metabolism in a Turkish patient [case report] PMID: 20127417
9. LMBRD1 is the gene underlying the cblF defect of cobalamin metabolism and suggests that LMBD1 is a lysosomal membrane exporter for cobalamin. PMID: 19136951
顯示更多

收起更多
相關(guān)疾病：

Methylmalonic aciduria and homocystinuria type cblF (MMAHCF)
亞細(xì)胞定位：

Endoplasmic reticulum membrane. Lysosome membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein. Cytoplasmic vesicle, clathrin-coated vesicle.
蛋白家族：

LIMR family, LMBRD1 subfamily
組織特異性：

Isoform 3 is expressed in liver.
數(shù)據(jù)庫(kù)鏈接：

HGNC: 23038

OMIM: 277380

KEGG: hsa:55788

STRING: 9606.ENSP00000359609

UniGene: Hs.271643

Most popular with customers

YWHAG Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
YWHAB Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF, FC

Species Reactivity: Human, Mouse, Rat
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
XRCC5 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF

Species Reactivity: Human
XPNPEP1 Recombinant Monoclonal Antibody

Applications: ELISA, IHC, FC

Species Reactivity: Human
XDH Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
WT1 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
WFDC2 Recombinant Monoclonal Antibody

Applications: ELISA, WB, FC

Species Reactivity: Human