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Rabbit Anti-MLC1  antibody (bs-7115R)  
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產品編號 bs-7115R
英文名稱 Rabbit Anti-MLC1  antibody
中文名稱 膜蛋白MLC1抗體
別    名 KIAA0027; LVM; Megalencephalic leukoencephalopathy with subcortical cysts 1; Membrane protein MLC1; MLC; MLC-1; MLC1_HUMAN; VL; WKL1.  
Specific References  (2)     |     bs-7115R has been referenced in 2 publications.
[IF=8.713] Zhao-Bo Luo. et al. Fecal transplant from myostatin deletion pigs positively impacts the gut-muscle axis. ELIFE. 2023; 12: e81858  WB ;  Mouse.  
[IF=5.168] Gu et al. Fasudil attenuates soluble fms-like tyrosine kinase-1 (sFlt-1)-induced hypertension in pregnant mice through RhoA/ROCK pathway. (2017) Oncotarget. 8:104104-104112  WB ;  Mouse.  
研究領域 細胞生物  神經生物學  信號轉導  通道蛋白  細胞膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,Rat (predicted: Human,Rabbit,Pig,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 41kDa
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MLC1: 321-377/377 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 MLC1 is a 377 amino acid multi-pass membrane protein that may serve as a non-selective neuronal cation channel in brain. Mutant MLC1 proteins that show impaired folding have been corrected in vitro with the addition of a Ca(2+)-ATPase inhibitor, curcumin. Mutations in the gene encoding MLC1 is the cause of megalencephalic leukoencephalopathy with subcortical cysts, also known as van der Knaap disease, a rare syndrome characterized early in life by progressive brain destruction causing mental retardation and incoordination. Single nucleotide polymorphisms within the MLC1 gene may be associated with periodic catatonia, but there seems to be conflicting evidence on whether or not the gene is implicated in general schizophrenia.

Function:
May be a transporter. May act as a non-selective neuronal cation channel.

Subunit:
Interacts with ATP1B1. Part of a complex containing ATP1B1, TRPV4, AQP4 and HEPACAM.

Subcellular Location:
Membrane; Multi-pass membrane protein (Potential). Cell membrane. Cytoplasm, perinuclear region. Endoplasmic reticulum.

Tissue Specificity:
Expressed in the brain, with highest levels found in the amygdala, nucleus caudatus, thalamus and hippocampus.

DISEASE:
Defects in MLC1 are a cause of leukoencephalopathy megalencephalic with subcortical cysts (MLC) [MIM:604004]. MLC is a syndrome of cerebral leukoencephalopathy and megalencephaly characterized by ataxia, spasticity, seizures, delay in motor development and mild mental retardation. The brain appears swollen on magnetic resonance imaging, with diffuse white-matter abnormalities and the invariable presence of subcortical cysts in frontal and temporal lobes.

SWISS:
Q15049

Gene ID:
23209

Database links:

Entrez Gene: 23209 Human

Entrez Gene: 170790 Mouse

Entrez Gene: 315215 Rat

Omim: 605908 Human

SwissProt: Q15049 Human

SwissProt: Q8VHK5 Mouse

Unigene: 517729 Human

Unigene: 32780 Mouse



產品圖片
Paraformaldehyde-fixed, paraffin embedded (rat placenta); 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; Incubation with (MLC1) Polyclonal Antibody, Unconjugated (bs-7115R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (mouse bone marrow); 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; Incubation with (MLC1) Polyclonal Antibody, Unconjugated (bs-7115R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (rat bone marrow); 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; Incubation with (MLC1) Polyclonal Antibody, Unconjugated (bs-7115R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB 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 (MLC1) Polyclonal Antibody, Unconjugated (bs-7115R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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