Dux Rabbit Polyclonal Antibody (ER1901-52)

UNIPROT #

Q9UBX2

PROTEIN NAME

Dux

SYNONYMS

Double homeobox 4 antibody;Double homeobox protein 10 antibody;Double homeobox protein 4 antibody;Double homeobox protein 4/10 antibody;DUX10 antibody;DUX4L antibody;DUX4L1 antibody

SEQUENCE SIMILARITIES

Belongs to the paired homeobox family.

TISSUE SPECIFICITY

Isoform 1: Does not seem to be expressed in normal muscle, but is detected in muscle of individuals with FSHD, and also in testis (at protein level). Isoform 1: Does not seem to be expressed in normal muscle, but in muscle of individuals with FSHD, where it may be toxic to cells. Isoform 2: Detected in skeletal muscle, fibroblasts and testis from healthy individuals.

DEVELOPMENTAL STAGE

Isoform 1: Detected in embryos at the 4-cell stage. Not detected in embryos at the 2-cell stage, or at the 8-cell stage (at protein level). Detected in embryos at the 4-cell stage. Not detected in embryos at the 2-cell stage, or at the 8-cell stage. Detected in induced pluripotent (iPS) cells, but expression is suppressed upon differentiation to embryoid bodies. Isoform 2: Detected in embryoid bodies derived from induced pluripotent (iPS) cells, but not in the induced pluripotent (iPS) cells themselves.

SUBCELLULAR LOCATION

Nucleus.

FUNCTION

The double homeobox (DUX) proteins are encoded by 3.3-kilobase repeats found throughout the human genome. The DUX family includes DUX1, DUX2, DUX3, DUX4 and DUX5. Each of these family members, excluding DUX2, contains two homeobox domains. DUX2 contains only one homeobox domain. DUX1 and DUX5 are identical to one another and they share 98% identity with DUX3 and approximately 70% identity with DUX2. The genes encoding DUX5 and DUX3 both contain additional start sites that result in N-terminal extended isoforms. The homeodomains found in DUX5 and DUX1 are similar to those found in Pax-3, Pax-7, OTX1 and OTX2. DUX4, also known as DUX10, is capable of forming homodimers. In addition, the gene encoding DUX4 maps within the D4Z4 repeat unit that has been implicated in Facioscapulohumeral muscular dystrophy (FSHD).