Product info:

This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also.

Alternative names:

OTC; OCTD

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Cellular localisation:

Mitochondrion matrix

Isotype:

IgG

Immunogen:

Recombinant fusion protein containing a sequence corresponding to amino acids 215-354 of human OTC (NP_000522.3).

Positive control:

Mouse liver, Mouse small intestine, Rat liver, Rat small intestine

AA Sequence:

LQAATPKGYEPDASVTKLAEQYAKENGTKLLLTNDPLEAAHGGNVLITDTWISMGQEEEKKKRLQAFQGYQVTMKTAKVAASDWTFLHCLPRKPEEVDDEVFYSPRSLVFPEAENRKWTIMAVMVSLLTDYSPQLQKPKF

Purification:

Affinity purification

Molecular weight:

Calculated MW: 39kDa Observed MW: 38kDa

Form:

Liquid

Applications:

Request info at info@sobekbio.com

Dilution:

WB 1:1000 - 1:4000 IHC 1:50 - 1:100

Storage buffer:

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

More info:

Email: info@sobekbio.com

Orders:

Email: orders@sobekbio.com