LMAN1 Antilichaam - N-terminaal gebied: Biotine (ARP46613_P050-Biotine)

Lectin, mannose-binding, 1

MR60, gp58, F5F8D, FMFD1, MCFD1, ERGIC53, ERGIC-53

3998

P49257

NP_005561

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Pig, Rabbit, Zebrafish

The immunogen is a synthetic peptide directed towards the N terminal region of human LMAN1

LMAN1 is a type I integral membrane protein localized in the intermediate region between the endoplasmic reticulum and the Golgi, presumably recycling between the two compartments. The protein is a mannose-specific lectin and is a member of a novel family of plant lectin homologs in the secretory pathway of animal cells. Mutations in its gene are associated with a coagulation defect. Using positional cloning, its gene was identified as the disease gene leading to combined factor V-factor VIII deficiency, a rare, autosomal recessive disorder in which both coagulation factors V and VIII are diminished.The protein encoded by this gene is a type I integral membrane protein localized in the intermediate region between the endoplasmic reticulum and the Golgi, presumably recycling between the two compartments. The protein is a mannose-specific lectin and is a member of a novel family of plant lectin homologs in the secretory pathway of animal cells. Mutations in the gene are associated with a coagulation defect. Using positional cloning, the gene was identified as the disease gene leading to combined factor V-factor VIII deficiency, a rare, autosomal recessive disorder in which both coagulation factors V and VIII are diminished. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.

UBC; NEDD8; YIPF3; env; PIGS; TMED2; YBX1; HSP90AA1; HNRNPU; APP; UBXN6; RAB3GAP2; RAB3GAP1; COPE; TUBB4B; TUBB3; COPB2; VCP; DNAJC7; P4HB; HSPA8; HSPA1A; COPB1; COPA; BCAP29; BCAP31; HLA-A; CANX; ELAVL1; MCFD2; F8

Biotin

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 100%; Dog: 100%; Guinea Pig: 93%; Horse: 100%; Human: 100%; Mouse: 100%; Pig: 100%; Rabbit: 93%; Rat: 100%; Zebrafish: 93%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

54kDa

Zhang, B., (er) Blood (2008) In press

Wet Ice

510

LMAN1

LMAN1

Rabbit

Protein ERGIC-53

NM_005570

DPAVALPHRRFEYKYSFKGPHLVQSDGTVPFWAHAGNAIPSSDQIRVAPS