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Anti-Dymeclin antibody (bs-13037R)

Anti-Dymeclin antibody (bs-13037R)

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Anti-Dymeclin antibody 

英文名称Dymeclin
中文名称迪格弗-梅尔基奥尔-克劳森综合征相关蛋白抗体
别    名DMC; Dyggve-Melchior-Clausen syndrome protein; DYM; FLJ20071; FLJ90130; SMC; DYM_HUMAN.  

DATASHEET

Host:Rabbit

Target Protein:Dymeclin

IR:Immunogen Range:151-250/669

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:54808

Swiss Prot:Q7RTS9

Source:KLH conjugated synthetic peptide derived from human Dymeclin:151-250/669 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Background:Dyggve-Melchior-Clausen syndrome (DMC), a rare autosomal recessive disorder, is characterized by microcephaly, short trunk dwarfism and sometime psychomotor retardation. Cutaneous cells of affected individuals show dilated rough endoplasmic reticulum and enlarged vacuoles. The Dyggve-Melchior-Clausen syndrome protein, also designated dymeclin, may play a role in proteoglycan metabolism and intracellular protein digestion. It is a widely expressed multi-pass membrane protein, detected primarily in chondrocytes and fetal brain tissue. Defects in dymeclin are also the cause of Smith-McCort dysplasis syndrome (SMC), which has characteristics identical to those of Dyggve-Melchior-Clausen syndrome.

Size:100ul

Concentration:1mg/ml

Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)

Cross Reactive Species:Human
Mouse
Rat
Dog
Cow
Horse
Sheep
.

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Tissue/cell: Human kidney 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- Dymeclin Polyclonal Antibody, Unconjugated(bs-13037R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining

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- Dymeclin Polyclonal Antibody, Unconjugated(bs-13037R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining

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