Description:

Size: 100ul

Catalog no.: bs-9178R-A647

Price: 380 EUR

Product details

Gene ID Number

4534

Target Antigen

MTM1

Modification Site

None

Tested applications

IF(IHC-P)

French translation

anticorps

Clonality

Polyclonal

Modification

Unmodified

Concentration

1ug per 1ul

Excitation emission

650nm/665nm

Conjugated with

ALEXA FLUOR® 647

Crossreactivity

Human, Mouse, Rat

Clone

Polyclonal antibody

Recommended dilutions

IF(IHC-P)(1:50-200)

Purification

Purified by Protein A.

Conjugation

Alexa Fluor,ALEXA FLUOR 647

Category

Conjugated Primary Antibodies

Also known as

Anti-MTM1 PAb ALEXA FLUOR 647

Host Organism

Rabbit (Oryctolagus cuniculus)

Specificity

This is a highly specific antibody against MTM1.

Long name

MTM1 Polyclonal Antibody, ALEXA FLUOR 647 Conjugated

Cross-reactive species details

Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.

Synonyms

CG2; CNM; KIAA4176; mKIAA4176; Mtm; Mtm1; MTM1_HUMAN; MTMX; Myotubular myopathy 1; Myotubularin; XLMTM.

Source

This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human MTM1/Myotubularin

Storage conditions

Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.

Properties

For facs or microscopy Alexa 1 conjugate.Alexa Fluor 633 is a practical alternative to APC as well as Cy5. Bioss Primary Conjugated Antibodies. ALEXA FLUOR made this Alexa Fluor 633 conjugate that can be used in multi-color flow cytometry with instruments equipped with a second red laser or red diode. It is detected in the FL4 detector of the core's upgraded 2-laser FACScans. Like other Alexa Fluor dyes, the Anti-MTM1 exhibits uncommon photo stability, making it an ideal choice for fluorescent microscopy.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.

Background of the antigen

X-linked recessive myotubular myopathy is a congenital muscular disease characterized by severe hypotonia and generalized muscle weakness that, in most cases, leads to early postnatal death. The gene responsible for myotubular myopathy MTM1 encodes a dual specificity phosphatase, named myotubularin, which is highly conserved through evolution. The gene for MTM1 is localized to a 300 kb critical region on human Xq128 between IDS and GRBRA3. Human MTM1, a 603 amino-acid protein, is mutated in myotubular myopathy. The largely related protein hMTMR2 is found mutated in a recessive form of Charcot-Marie-Tooth neuropathy. Myotubularin is primarily a lipid phosphatase that acts on phosphatidylinositol 3-monophosphate and is involved in the regulation of the phosphatidylinositol 3-kinase (PI3-kinase) pathway and membrane trafficking. Wild-type myotubularin can directly dephosphorylate PI3P and PI4P in vitro. Thus, it decreases PI3P levels by down-regulating PI3K activity and by facilitating the degradation of PI3P.