Anti-Aspartate beta hydroxylase/ASPH Antibody Picoband® Fluoro647 Conjugated

ASPH is also known as Aspartyl/asparaginyl beta-hydroxylase. This gene is thought to play an important role in calcium homeostasis. And the gene is expressed from two promoters and undergoes extensive alternative splicing. The encoded set of proteins share varying amounts of overlap near their N-termini but have substantial variations in their C-terminal domains resulting in distinct functional properties. The longest isoforms (a and f) include a C-terminal Aspartyl/Asparaginyl beta-hydroxylase domain that hydroxylates aspartic acid or asparagine residues in the epidermal growth factor (EGF) -like domains of some proteins, including protein C, coagulation factors VII, IX, and X, and the complement factors C1R and C1S. Other isoforms differ primarily in the C-terminal sequence and lack the hydroxylase domain, and some have been localized to the endoplasmic and sarcoplasmic reticulum. Some of these isoforms are found in complexes with calsequestrin, triadin, and the ryanodine receptor, and have been shown to regulate calcium release from the sarcoplasmic reticulum. Some isoforms have been implicated in metastasis.

Isoform 1 is detected in all tissues tested. Isoform 8 is mainly expressed in pancreas, heart, brain, kidney and liver. Isoform 8 is expressed in kidney (at protein level) . .

1. Entrez Gene: ASPH aspartate beta-hydroxylase. 2. Korioth F, Gieffers C, Frey J (Feb 1995) . Cloning and characterization of the human gene encoding aspartyl beta-hydroxylase. Gene 150 (2) : 395–9. 3. Lim KY, Hong CS, Kim DH (Nov 2000) . cDNA cloning and characterization of human cardiac junctin. Gene 255 (1) : 35–42.