SUMF1 Polyklonale Antilichaam, AbBy FluorTM 647 Vervoeging

SUMF1 is a 374 amino acid alternatively spliced protein that localizes to the lumen of the endoplasmic reticulum and belongs to the sulfatase-modifying factor family. Expressed ubiquitously with highest expression in liver, kidney and pancreas, SUMF1 exists as either a monomer, a homodimer or a heterodimer (with SUMF2) and functions to oxidize sulfatase cysteine residues to an active FGIy residue, thereby playing an important role in sulfatase activity. Defects in the gene encoding SUMF1 are the cause of multiple sulfatase deficiency (MSD), a heterogeneous disorder characterized by metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.

MGC150436; AAPA3037; C alpha formylglycine generating enzyme 1; C-alpha-formylglycine-generating enzyme 1; FGE; FGly generating enzyme; MGC131853; Sulfatase modying factor 1 [Precursor]; Sulfatase-modying factor 1; SUMF1; SUMF1_HUMAN; UNQ3037.

285362

Cytoplasm

301-374/374

SUMF1

AbBy Fluor™ 647

KLH conjugated synthetic peptide derived from human SUMF1

WB, IF (IHC-P), IF (IHC-F), IF (ICC)

Purified by Protein A.

650nm/665nm

1µg/µl

WB (1:300-5000), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)

Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Unmodified

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

285362

Human, Mouse, Rat, Dog, Cow, Sheep, Pig, Horse, Rabbit